PMID-sentid	Pub_year	Sent_text	compound_name	comp_offset	prot_official_name	organism	prot_offset
33924567-2	2021	Fabry disease (FD) is a lysosomal storage disorder caused by deficient alpha-galactosidase A activity in the lysosome due to mutations in the GLA gene, resulting in gradual accumulation of globotriaosylceramide and other derivatives in different tissues.	globotriaosylceramide	189-210	galactosidase alpha	Homo sapiens	71-92
33924567-2	2021	Fabry disease (FD) is a lysosomal storage disorder caused by deficient alpha-galactosidase A activity in the lysosome due to mutations in the GLA gene, resulting in gradual accumulation of globotriaosylceramide and other derivatives in different tissues.	globotriaosylceramide	189-210	galactosidase alpha	Homo sapiens	142-145
33555391-2	2021	Our previous work identified the interaction between the bacterial lectin LecA and its host cell glycosphingolipid receptor globotriaosylceramide (Gb3) as a crucial step for the engulfment of P. aeruginosa via the lipid zipper mechanism.	globotriaosylceramide	124-145	PA-I galactophilic lectin	Pseudomonas aeruginosa PAO1	74-78
33968642-2	2021	Tissue and organ changes are caused by widespread progressive accumulation of globotriaosylceramide (Gb3) and globotriaosylsphingosine (lysoGb3).	globotriaosylceramide	78-99	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	101-104
33968643-2	2021	The deposition of globotriaosylceramide (Gb3) may cause damage to all organs, particularly brain, heart and kidney.	globotriaosylceramide	18-39	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	41-44
33725118-1	2021	AIMS: Fabry disease (FD) is an X-linked lysosomal storage disorder caused by a deficiency of the lysosomal enzyme alpha-galactosidase A (GLA/AGAL) resulting in lysosomal accumulation of globotriaosylceramide (Gb3).	globotriaosylceramide	186-207	galactosidase alpha	Homo sapiens	114-135
33910691-1	2021	Fabry disease is due to mutations in the GLA gene that cause a deficiency of the activity of the lysosomal enzyme alpha-galactosidase A (alpha-gal A) resulting in intra-tissue accumulation of globotriaosylceramide.	globotriaosylceramide	192-213	galactosidase alpha	Homo sapiens	41-44
33910691-1	2021	Fabry disease is due to mutations in the GLA gene that cause a deficiency of the activity of the lysosomal enzyme alpha-galactosidase A (alpha-gal A) resulting in intra-tissue accumulation of globotriaosylceramide.	globotriaosylceramide	192-213	galactosidase alpha	Homo sapiens	114-135
33910691-1	2021	Fabry disease is due to mutations in the GLA gene that cause a deficiency of the activity of the lysosomal enzyme alpha-galactosidase A (alpha-gal A) resulting in intra-tissue accumulation of globotriaosylceramide.	globotriaosylceramide	192-213	galactosidase alpha	Homo sapiens	137-148
33910691-3	2021	It binds, in a specific and reversible manner, to the catalytic site of alpha-gal A mutants, to prevent their degradation by the quality control system of the endoplasmic reticulum and allow them to catabolize globotriaosylceramide in the lysosomes.	globotriaosylceramide	210-231	galactosidase alpha	Homo sapiens	72-83
33765701-1	2021	Fabry disease is a rare X-linked genetic lysosomal storage disorder caused by mutations in the GLA gene, which results of reduced or absent activity of alpha-galactosidase A, accumulation of metabolic substrates globotriaosylceramide (GL-3) and derivatives deacylated derivative globotriaosylsphingosine (Lyso-GL-3) in multiple tissues, and multi-organ diseases and even life-threatening complications.	globotriaosylceramide	212-233	galactosidase alpha	Homo sapiens	95-98
33661535-2	2021	Deficiency of the lysosomal enzyme alpha-galactosidase (GLA) leads to accumulation of potentially toxic globotriaosylceramide (Gb3) on a multisystem level.	globotriaosylceramide	104-125	galactosidase alpha	Homo sapiens	56-59
33661535-2	2021	Deficiency of the lysosomal enzyme alpha-galactosidase (GLA) leads to accumulation of potentially toxic globotriaosylceramide (Gb3) on a multisystem level.	globotriaosylceramide	104-125	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	127-130
33477028-1	2021	Identification of 19 molecular species of globotriaosylceramides (Gb3) in extracts from a Fabry"s plasma patient and a healthy control was performed by High-Performance Thin-Layer Chromatography (HPTLC)-densitometry and online coupling to Mass Spectrometry (MS).	globotriaosylceramide	42-64	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	66-69
33531072-1	2021	BACKGROUND: Fabry disease (FD) is a rare X-linked disease caused by mutations in GLA gene with consequent lysosomal accumulation of globotriaosylceramide (Gb3).	globotriaosylceramide	132-153	galactosidase alpha	Homo sapiens	81-84
33531072-1	2021	BACKGROUND: Fabry disease (FD) is a rare X-linked disease caused by mutations in GLA gene with consequent lysosomal accumulation of globotriaosylceramide (Gb3).	globotriaosylceramide	132-153	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	155-158
32948848-1	2021	Fabry is an X-linked disorder of glycosphingolipid metabolism that is caused by variants of the GLA gene that codes for alpha-galactosidase A, leading to lysosomal accumulation of globotriaosylceramide in many cell types.	globotriaosylceramide	180-201	galactosidase alpha	Homo sapiens	96-99
32948848-1	2021	Fabry is an X-linked disorder of glycosphingolipid metabolism that is caused by variants of the GLA gene that codes for alpha-galactosidase A, leading to lysosomal accumulation of globotriaosylceramide in many cell types.	globotriaosylceramide	180-201	galactosidase alpha	Homo sapiens	120-141
33572752-5	2021	Indeed, mutations of the galactosidase alpha gene (GLA) cause a reduction or lack of GAL activity leading to globotriaosylceramide (Gb3) accumulation in several organs.	globotriaosylceramide	109-130	galactosidase alpha	Homo sapiens	25-44
33572752-5	2021	Indeed, mutations of the galactosidase alpha gene (GLA) cause a reduction or lack of GAL activity leading to globotriaosylceramide (Gb3) accumulation in several organs.	globotriaosylceramide	109-130	galactosidase alpha	Homo sapiens	51-54
33572752-5	2021	Indeed, mutations of the galactosidase alpha gene (GLA) cause a reduction or lack of GAL activity leading to globotriaosylceramide (Gb3) accumulation in several organs.	globotriaosylceramide	109-130	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	132-135
33495303-1	2022	BACKGROUND: Fabry disease (FD) is a rare X-linked lysosomal storage disease caused by mutations in the alpha-galactosidase A gene (GLA) leading to deficiency of alpha-galactosidase A and ultimately in progressive glycosphingolipid accumulation, especially globotriaosylceramide (Gb3) and its deacylated derivative globotriaosylsphingosine (Lyso-Gb3).	globotriaosylceramide	256-277	galactosidase alpha	Homo sapiens	131-134
33721270-2	2021	The lysosomal accumulation of glycosphingolipids, especially globotriaosylceramide (Gb3) and globotriaosylsphingosine (lyso-Gb3, deacylated form), leads to a multisystemic disease with progressive renal failure, cardiomyopathy with potentially malignant cardiac arrhythmias, and strokes, which considerably limits the life expectancy of affected patients.	globotriaosylceramide	61-82	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	84-87
33592213-6	2021	RESULTS: Total and subspecies of serum SMs and globotriaosyl ceramides (Gb3s) were positively related to GIR30, free FAs (FFA 16:1, FFA20:4), some long chain GM3 and complex ceramide GluCers showed strong negative correlations with GIR30.	globotriaosylceramide	47-70	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	72-76
33738082-7	2021	Moreover, a single intravenous administration of EV-GLA was able to reduce globotriaosylceramide (Gb3) substrate levels in clinically relevant tissues, such kidneys and brain.	globotriaosylceramide	75-96	galactosidase alpha	Homo sapiens	52-55
33738082-7	2021	Moreover, a single intravenous administration of EV-GLA was able to reduce globotriaosylceramide (Gb3) substrate levels in clinically relevant tissues, such kidneys and brain.	globotriaosylceramide	75-96	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	98-101
33673160-1	2021	Fabry disease (FD) is a lysosomal storage disorder (LSD) characterized by the deficiency of alpha-galactosidase A (alpha-GalA) and the consequent accumulation of toxic metabolites such as globotriaosylceramide (Gb3) and globotriaosylsphingosine (lysoGb3).	globotriaosylceramide	188-209	galactosidase alpha	Homo sapiens	115-125
33725118-1	2021	AIMS: Fabry disease (FD) is an X-linked lysosomal storage disorder caused by a deficiency of the lysosomal enzyme alpha-galactosidase A (GLA/AGAL) resulting in lysosomal accumulation of globotriaosylceramide (Gb3).	globotriaosylceramide	186-207	galactosidase alpha	Homo sapiens	137-140
33577039-2	2021	This is caused by pathogenic variants in the GLA gene, coding for the lysosomal enzyme called alpha-galactosidase A (aGLA), responsible for the cleavage of globotriaosylceramide (Gb3).	globotriaosylceramide	156-177	galactosidase alpha	Homo sapiens	45-48
33156427-1	2021	Fabry disease (FD) is a rare X-linked glycosphingolipidosis caused by mutations in GLA, a gene responsible for encoding alpha-galactosidase A, an enzyme required for degradation of glycosphingolipids, mainly globotriaosylceramide (Gb3) in all cells of the body.	globotriaosylceramide	208-229	galactosidase alpha	Homo sapiens	83-86
33156427-1	2021	Fabry disease (FD) is a rare X-linked glycosphingolipidosis caused by mutations in GLA, a gene responsible for encoding alpha-galactosidase A, an enzyme required for degradation of glycosphingolipids, mainly globotriaosylceramide (Gb3) in all cells of the body.	globotriaosylceramide	208-229	galactosidase alpha	Homo sapiens	120-141
33577039-2	2021	This is caused by pathogenic variants in the GLA gene, coding for the lysosomal enzyme called alpha-galactosidase A (aGLA), responsible for the cleavage of globotriaosylceramide (Gb3).	globotriaosylceramide	156-177	galactosidase alpha	Homo sapiens	94-115
33577039-2	2021	This is caused by pathogenic variants in the GLA gene, coding for the lysosomal enzyme called alpha-galactosidase A (aGLA), responsible for the cleavage of globotriaosylceramide (Gb3).	globotriaosylceramide	156-177	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	179-182
32994552-4	2021	The relationship between changes in lyso-Gb3 and kidney interstitial capillary (KIC) globotriaosylceramide (Gb3) inclusions was assessed in treatment-naive patients.	globotriaosylceramide	85-106	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	41-44
33460651-2	2021	It synthesizes the Galalpha1 4Gal linkage on two different glycosphingolipids (GSLs), producing globotriaosylceramide (Gb3, CD77, Pk) and the P1 antigen.	globotriaosylceramide	96-117	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	119-122
32868283-1	2020	Fabry disease is caused by deficient activity of alpha-galactosidase A-an enzyme that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins--and subsequent accumulation of glycosphingolipids, mainly globotriaosylceramide (Gb3), globotriaosylsphingosine (lyso-Gb3) and galabiosylceramide.	globotriaosylceramide	230-251	galactosidase, alpha	Mus musculus	49-70
33460651-2	2021	It synthesizes the Galalpha1 4Gal linkage on two different glycosphingolipids (GSLs), producing globotriaosylceramide (Gb3, CD77, Pk) and the P1 antigen.	globotriaosylceramide	96-117	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	124-128
32772454-5	2020	Macrophage-like differentiated THP-1 cells treated with Stxs secreted Stx-associated exosomes (Stx-Exo) of 90-130 nm in diameter, which induced cytotoxicity in recipient cells in a toxin receptor globotriaosylceramide (Gb3 )-dependent manner.	globotriaosylceramide	196-217	ST8 alpha-N-acetyl-neuraminide alpha-2,8-sialyltransferase 2	Homo sapiens	56-59
32772454-5	2020	Macrophage-like differentiated THP-1 cells treated with Stxs secreted Stx-associated exosomes (Stx-Exo) of 90-130 nm in diameter, which induced cytotoxicity in recipient cells in a toxin receptor globotriaosylceramide (Gb3 )-dependent manner.	globotriaosylceramide	196-217	ST8 alpha-N-acetyl-neuraminide alpha-2,8-sialyltransferase 2	Homo sapiens	70-73
32661622-1	2020	Fabry disease (FD) is a multisystemic X-linked disorder characterized by the accumulation of lysosomal globotriaosylceramide (Gb3) secondary to decreased activity of alpha-galactosidase in cells.	globotriaosylceramide	103-124	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	126-129
32854306-2	2020	This enzyme cleaves the last sugar unit of glycosphingolipids, including globotriaosylceramide (Gb3), globotriaosylsphingosine (lyso-Gb3), and galabiosylceramide (Ga2).	globotriaosylceramide	73-94	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	96-99
32738442-1	2020	Fabry disease is an X-linked lysosomal storage disorder caused by the deficiency of the enzyme, alpha-galactosidase A that induces the accumulation of the substrate globotriaosylceramide.	globotriaosylceramide	165-186	galactosidase alpha	Homo sapiens	96-117
32248228-2	2020	Silurus asotus egg lectin (SAL) has been reported to enhance the incorporation of propidium iodide as well as doxorubicin into Burkitt"s lymphoma Raji cells through binding to globotriaosylceramide (Gb3) on the cell surface.	globotriaosylceramide	176-197	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	199-202
32963035-1	2021	INTRODUCTION: Recent studies showed the usefulness of globotriaosylsphingosine (lyso-Gb3) and related analogues, deacylated forms of globotriaosylceramide (Gb3), for high-risk screening, treatment monitoring and follow-up for patients with Fabry disease.	globotriaosylceramide	133-154	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	85-88
33205006-5	2020	We report herewith that globotriaosylceramide (Gb3) is associated with cSrc kinase in GEMs and plays a crucial role in modulating expression of p53 R273H mutant and drug resistance.	globotriaosylceramide	24-45	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	47-50
33205006-5	2020	We report herewith that globotriaosylceramide (Gb3) is associated with cSrc kinase in GEMs and plays a crucial role in modulating expression of p53 R273H mutant and drug resistance.	globotriaosylceramide	24-45	SRC proto-oncogene, non-receptor tyrosine kinase	Homo sapiens	71-75
33205006-5	2020	We report herewith that globotriaosylceramide (Gb3) is associated with cSrc kinase in GEMs and plays a crucial role in modulating expression of p53 R273H mutant and drug resistance.	globotriaosylceramide	24-45	tumor protein p53	Homo sapiens	144-147
32699723-2	2020	This deficiency results in a progressive, multiorgan accumulation of glycolipids, most notably globotriaosylceramide (Gb3), leading to multiorgan failure and subsequently premature death.	globotriaosylceramide	95-116	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	118-121
32198894-1	2020	Fabry disease (FD) is an X-linked lysosomal storage disorder caused by the deficient activity of the lysosomal enzyme alpha-galactosidase A (alpha-Gal A) leading to intracellular accumulation of globotriaosylceramide (Gb3).	globotriaosylceramide	195-216	galactosidase alpha	Homo sapiens	118-139
32198894-1	2020	Fabry disease (FD) is an X-linked lysosomal storage disorder caused by the deficient activity of the lysosomal enzyme alpha-galactosidase A (alpha-Gal A) leading to intracellular accumulation of globotriaosylceramide (Gb3).	globotriaosylceramide	195-216	galactosidase alpha	Homo sapiens	141-152
32198894-1	2020	Fabry disease (FD) is an X-linked lysosomal storage disorder caused by the deficient activity of the lysosomal enzyme alpha-galactosidase A (alpha-Gal A) leading to intracellular accumulation of globotriaosylceramide (Gb3).	globotriaosylceramide	195-216	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	218-221
32673369-7	2020	We used the portable real-time optical sensing system to detect Alexa Fluor 488-tagged Stx2B-subunits bound to monocytic THP-1 cells expressing the toxin receptor globotriaosylceramide (Gb3).	globotriaosylceramide	163-184	syntaxin 2	Homo sapiens	87-92
32673369-7	2020	We used the portable real-time optical sensing system to detect Alexa Fluor 488-tagged Stx2B-subunits bound to monocytic THP-1 cells expressing the toxin receptor globotriaosylceramide (Gb3).	globotriaosylceramide	163-184	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	186-189
32189245-1	2020	Fabry disease is an X-linked inherited lysosomal storage disorder caused by a deficiency of alpha-galactosidase A activity, resulting in the intracellular accumulation of globotriaosylceramide and related glycosphingolipids.	globotriaosylceramide	171-192	galactosidase alpha	Homo sapiens	92-113
32606714-1	2020	Fabry disease (FD) is an X-linked lysosomal storage disorder caused by absence or deficient activity of alpha-galactosidase A (alpha-Gal A) due to mutations in the alpha-galactosidase A gene (GLA), leading to progressive accumulation of globotriaosylceramide (Gb3) in tissues and organs including heart, kidney, the eyes, vascular endothelium, the nervous system and the skin.	globotriaosylceramide	237-258	galactosidase alpha	Homo sapiens	104-125
32775495-2	2020	Deficient alpha-Gal A activity results in the progressive, systemic accumulation of its substrates, globotriaosylceramide (Gb3) and globotriaosylsphingosine (Lyso-Gb3), leading to renal, cardiac, and/or cerebrovascular disease and early demise.	globotriaosylceramide	100-121	galactosidase alpha	Homo sapiens	10-21
32775495-2	2020	Deficient alpha-Gal A activity results in the progressive, systemic accumulation of its substrates, globotriaosylceramide (Gb3) and globotriaosylsphingosine (Lyso-Gb3), leading to renal, cardiac, and/or cerebrovascular disease and early demise.	globotriaosylceramide	100-121	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	123-126
32640076-1	2020	Fabry disease (FD) is an X-linked lysosomal storage disorder caused by pathogenic variants in the alpha-galactosidase A gene (GLA) that leads to reduced or undetectable alpha-galactosidase A (AGAL-A) enzyme activity and progressive accumulation of globotriaosylceramide (Gb3 ) and its deacylated form globotriaosylsphingosine (lysoGb3 ) in cells throughout the body.	globotriaosylceramide	248-269	galactosidase alpha	Homo sapiens	126-129
32640076-1	2020	Fabry disease (FD) is an X-linked lysosomal storage disorder caused by pathogenic variants in the alpha-galactosidase A gene (GLA) that leads to reduced or undetectable alpha-galactosidase A (AGAL-A) enzyme activity and progressive accumulation of globotriaosylceramide (Gb3 ) and its deacylated form globotriaosylsphingosine (lysoGb3 ) in cells throughout the body.	globotriaosylceramide	248-269	galactosidase alpha	Homo sapiens	98-119
32389574-1	2020	Fabry disease is a rare X-linked lysosomal disease, in which mutations in the gene encoding alpha-galactosidase A result in progressive cellular accumulation of globotriaosylceramide (GL-3) in various organs including the skin, kidney, and heart, often leading to life-threatening conditions.	globotriaosylceramide	161-182	galactosidase alpha	Homo sapiens	92-113
32606714-1	2020	Fabry disease (FD) is an X-linked lysosomal storage disorder caused by absence or deficient activity of alpha-galactosidase A (alpha-Gal A) due to mutations in the alpha-galactosidase A gene (GLA), leading to progressive accumulation of globotriaosylceramide (Gb3) in tissues and organs including heart, kidney, the eyes, vascular endothelium, the nervous system and the skin.	globotriaosylceramide	237-258	galactosidase alpha	Homo sapiens	127-138
32606714-1	2020	Fabry disease (FD) is an X-linked lysosomal storage disorder caused by absence or deficient activity of alpha-galactosidase A (alpha-Gal A) due to mutations in the alpha-galactosidase A gene (GLA), leading to progressive accumulation of globotriaosylceramide (Gb3) in tissues and organs including heart, kidney, the eyes, vascular endothelium, the nervous system and the skin.	globotriaosylceramide	237-258	galactosidase alpha	Homo sapiens	164-185
32606714-1	2020	Fabry disease (FD) is an X-linked lysosomal storage disorder caused by absence or deficient activity of alpha-galactosidase A (alpha-Gal A) due to mutations in the alpha-galactosidase A gene (GLA), leading to progressive accumulation of globotriaosylceramide (Gb3) in tissues and organs including heart, kidney, the eyes, vascular endothelium, the nervous system and the skin.	globotriaosylceramide	237-258	galactosidase alpha	Homo sapiens	192-195
32532136-2	2020	This disorder leads to intracellular globotriaosylceramide (Gb3) deposition associated with increased Gb3 plasma levels.	globotriaosylceramide	37-58	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	60-63
32532136-2	2020	This disorder leads to intracellular globotriaosylceramide (Gb3) deposition associated with increased Gb3 plasma levels.	globotriaosylceramide	37-58	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	102-105
32087678-2	2020	BACKGROUND: Fabry disease (FD) is a rare, lysosomal storage disorder caused by the absence or deficiency of the enzyme alpha-galactosidase A (alpha-Gal A) that leads to the abnormal accumulation of the lipid globotriaosylceramide (GB3) in a variety of cell types and tissues throughout the body.	globotriaosylceramide	208-229	galactosidase alpha	Homo sapiens	119-140
32523894-3	2020	The aim of this study was to examine if recipient cells must express the globotriaosylceramide (Gb3) toxin receptor for this to occur, or if Gb3-negative cells are also susceptible after uptake of Gb3-positive and toxin-positive microvesicles.	globotriaosylceramide	73-94	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	96-99
32296648-2	2020	This AB5 subunit toxin family bind target cell globotriaosyl ceramide (Gb3), a glycosphingolipid (GSL) (aka CD77, pk blood group antigen) of the globoseries of neutral GSLs, initiating lipid raft-dependent plasma membrane Gb3 clustering, membrane curvature, invagination, scission, endosomal trafficking, and retrograde traffic via the TGN to the Golgi, and ER.	globotriaosylceramide	47-69	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	108-112
32183018-3	2020	Globotriaosylsphingosine (LysoGb3), the deacylated form of globotriaosylceramide (Gb3), is described as a highly sensitive biomarker for another lysosomal storage disease-Fabry disease.	globotriaosylceramide	59-80	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	30-33
32127409-2	2020	Defects in the gene encoding alpha-galactosidase A lead to accumulation of globotriaosylceramide (GL3) in various cell types.	globotriaosylceramide	75-96	galactosidase, alpha	Mus musculus	29-50
32292674-2	2020	This leads to intracellular accumulation of glycosphingolipids, mainly globotriaosylceramide (Gb3), throughout the body.	globotriaosylceramide	71-92	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	94-97
32087678-2	2020	BACKGROUND: Fabry disease (FD) is a rare, lysosomal storage disorder caused by the absence or deficiency of the enzyme alpha-galactosidase A (alpha-Gal A) that leads to the abnormal accumulation of the lipid globotriaosylceramide (GB3) in a variety of cell types and tissues throughout the body.	globotriaosylceramide	208-229	galactosidase alpha	Homo sapiens	142-153
31778673-7	2020	Globotriaosylceramide (Gb3) was analyzed in dried urine spots by liquid chromatography/tandem mass spectrometry followed by globotriaosylsphingosine (lyso-Gb3) on the repeat analysis.	globotriaosylceramide	0-21	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	23-26
31566927-2	2019	The GLA gene variant c.352C>T/p.Arg118Cys was considered with uncertain pathogenicity because of the finding of high residual alpha-galactosidase A (alpha-Gal A) enzyme activity, the absence of Mendelian segregation with an FD phenotype with many individuals remaining asymptomatic at old ages and the lack of globotriaosylceramide (Gb3) deposits in tissues.	globotriaosylceramide	310-331	galactosidase alpha	Homo sapiens	4-7
31778662-0	2020	Globotriaosylceramide-induced reduction of KCa1.1 channel activity and activation of the Notch1 signaling pathway in skin fibroblasts of male Fabry patients with pain.	globotriaosylceramide	0-21	potassium calcium-activated channel subfamily M alpha 1	Homo sapiens	43-49
31778662-0	2020	Globotriaosylceramide-induced reduction of KCa1.1 channel activity and activation of the Notch1 signaling pathway in skin fibroblasts of male Fabry patients with pain.	globotriaosylceramide	0-21	notch receptor 1	Homo sapiens	89-95
31939530-3	2020	Mutations in the GLA gene lead to the progressive accumulation of globotriaosylceramide (Gb3).	globotriaosylceramide	66-87	galactosidase alpha	Homo sapiens	17-20
31939530-3	2020	Mutations in the GLA gene lead to the progressive accumulation of globotriaosylceramide (Gb3).	globotriaosylceramide	66-87	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	89-92
32306353-3	2020	The StxB pentamer specifically recognizes a glycosphingolipid, globotriaosylceramide (Gb3), as a receptor; therefore, it can be used as a probe to detect Gb3.	globotriaosylceramide	63-84	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	86-89
32306353-3	2020	The StxB pentamer specifically recognizes a glycosphingolipid, globotriaosylceramide (Gb3), as a receptor; therefore, it can be used as a probe to detect Gb3.	globotriaosylceramide	63-84	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	154-157
31630715-1	2019	Anderson-Fabry Disease (AFD) is a rare, X-linked inborn error of glycosphingolipid catabolism caused by a deficient or absent activity of the lysosomal enzyme, alpha-galactosidase A, resulting in the progressive multisystem lysosomal accumulation of glycosphingolipids, mainly globotriaosylceramide (Gb3).	globotriaosylceramide	277-298	galactosidase alpha	Homo sapiens	160-181
31101366-0	2019	Renal globotriaosylceramide facilitates tubular albumin absorption and its inhibition protects against acute kidney injury.	globotriaosylceramide	6-27	albumin	Mus musculus	48-55
30413389-4	2018	As previously observed with siRNA knockdown of GLA, globotriaosylceramide (Gb3) accumulated in EA.hy926 cells.	globotriaosylceramide	52-73	galactosidase alpha	Homo sapiens	47-50
31291414-1	2019	BACKGROUND: Fabry disease (FD) is an X-linked lysosomal storage disorder caused by mutations in the alpha galactosidase A gene (GLA) that lead to the enzymatic deficiency of alpha galactosidase (alpha-Gal A), resulting in the accumulation of globotriaosylceramide (Gb3) and globotriaosylsphingosine (lyso-Gb3), causing multiple organ dysfunctions.	globotriaosylceramide	242-263	galactosidase alpha	Homo sapiens	100-121
31291414-1	2019	BACKGROUND: Fabry disease (FD) is an X-linked lysosomal storage disorder caused by mutations in the alpha galactosidase A gene (GLA) that lead to the enzymatic deficiency of alpha galactosidase (alpha-Gal A), resulting in the accumulation of globotriaosylceramide (Gb3) and globotriaosylsphingosine (lyso-Gb3), causing multiple organ dysfunctions.	globotriaosylceramide	242-263	galactosidase alpha	Homo sapiens	128-131
31291414-1	2019	BACKGROUND: Fabry disease (FD) is an X-linked lysosomal storage disorder caused by mutations in the alpha galactosidase A gene (GLA) that lead to the enzymatic deficiency of alpha galactosidase (alpha-Gal A), resulting in the accumulation of globotriaosylceramide (Gb3) and globotriaosylsphingosine (lyso-Gb3), causing multiple organ dysfunctions.	globotriaosylceramide	242-263	galactosidase alpha	Homo sapiens	195-206
31123589-2	2019	This enzymatic deficit results in the cellular accumulation of globotriaosylceramide (GL-3 or Gb3) and related glycosphingolipids in practically all organs and tissues in the body.	globotriaosylceramide	63-84	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	94-97
30972193-2	2019	The mutations lead to lack of or faulty enzyme causing accumulation of globotriaosylceramide (Gb3) and related glycosphingolipids including globotriaosylsphingosine (lyso-Gb3).	globotriaosylceramide	71-92	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	94-97
30972193-2	2019	The mutations lead to lack of or faulty enzyme causing accumulation of globotriaosylceramide (Gb3) and related glycosphingolipids including globotriaosylsphingosine (lyso-Gb3).	globotriaosylceramide	71-92	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	171-174
30853972-1	2019	Fabry disease (FD) is a rare X-linked alpha-galactosidase A (GLA) deficiency, resulting in progressive lysosomal accumulation of globotriaosylceramide (Gb3) in a variety of cell types.	globotriaosylceramide	129-150	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	152-155
30578766-5	2019	Furthermore, glycosphingolipids (particularly globotriaosylceramide) generated from serial ceramide glycosylation were seen to activate cSrc and beta-catenin signaling so as to upregulate METTL3 expression, in turn promoting expression of p53 R273H mutant protein, with consequent drug resistance.	globotriaosylceramide	46-67	SRC proto-oncogene, non-receptor tyrosine kinase	Homo sapiens	136-140
30578766-5	2019	Furthermore, glycosphingolipids (particularly globotriaosylceramide) generated from serial ceramide glycosylation were seen to activate cSrc and beta-catenin signaling so as to upregulate METTL3 expression, in turn promoting expression of p53 R273H mutant protein, with consequent drug resistance.	globotriaosylceramide	46-67	catenin beta 1	Homo sapiens	145-157
30578766-5	2019	Furthermore, glycosphingolipids (particularly globotriaosylceramide) generated from serial ceramide glycosylation were seen to activate cSrc and beta-catenin signaling so as to upregulate METTL3 expression, in turn promoting expression of p53 R273H mutant protein, with consequent drug resistance.	globotriaosylceramide	46-67	methyltransferase 3, N6-adenosine-methyltransferase complex catalytic subunit	Homo sapiens	188-194
30578766-5	2019	Furthermore, glycosphingolipids (particularly globotriaosylceramide) generated from serial ceramide glycosylation were seen to activate cSrc and beta-catenin signaling so as to upregulate METTL3 expression, in turn promoting expression of p53 R273H mutant protein, with consequent drug resistance.	globotriaosylceramide	46-67	tumor protein p53	Homo sapiens	239-242
30660999-2	2019	To address this knowledge disparity, we focused on biosynthesis of globotriaosylceramide (Gb3), the Shiga toxin (STx) receptor, and performed a genome-wide CRISPR/CAS9 knockout screen in HeLa cells using STx1-mediated cytotoxicity.	globotriaosylceramide	67-88	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	90-93
29979634-8	2019	With respect to the kidney and heart, we found that both organs accumulate alpha-Gal A substrates, including the established biomarkers, globotriaosylceramide and globotriaosylsphingosine.	globotriaosylceramide	137-158	galactosidase, alpha	Rattus norvegicus	75-86
29875425-0	2019	Identification of lysosomal and extralysosomal globotriaosylceramide (Gb3) accumulations before the occurrence of typical pathological changes in the endomyocardial biopsies of Fabry disease patients.	globotriaosylceramide	47-68	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	70-73
29875425-3	2019	We aimed to examine globotriaosylceramide (Gb3) deposits in patients" endomyocardial biopsies to understand the early pathogenesis of FD cardiomyopathy.	globotriaosylceramide	20-41	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	43-46
30117110-2	2019	Mutations in the gene coding for alpha-galactosidase A lead to globotriaosylceramide (Gb-3) accumulation in lysosomes and in placenta and umbilical cord.	globotriaosylceramide	63-84	galactosidase alpha	Homo sapiens	33-54
30117110-2	2019	Mutations in the gene coding for alpha-galactosidase A lead to globotriaosylceramide (Gb-3) accumulation in lysosomes and in placenta and umbilical cord.	globotriaosylceramide	86-90	galactosidase alpha	Homo sapiens	33-54
31242288-1	2019	Purpose: Fabry disease (FD) is a multiorgan X-linked condition characterized by a deficiency of the lysosomal enzyme alpha-galactosidase A, resulting in a progressive intralysosomal deposit of globotriaosylceramide.	globotriaosylceramide	193-214	galactosidase alpha	Homo sapiens	117-138
30965672-3	2019	It is well documented that alpha-galactosidase A (GLA) enzyme activity deficiency causes globotriaosylceramide (Gb3) accumulation, which plays a crucial role in the etiology of FD.	globotriaosylceramide	89-110	galactosidase alpha	Homo sapiens	27-48
30965672-3	2019	It is well documented that alpha-galactosidase A (GLA) enzyme activity deficiency causes globotriaosylceramide (Gb3) accumulation, which plays a crucial role in the etiology of FD.	globotriaosylceramide	89-110	galactosidase alpha	Homo sapiens	50-53
30632067-4	2019	We could show that the presence of tumor-derived mHsp70 in TNTs with a diameter ranging from 120 to 140 nm predominantly originates from cholesterol-rich-microdomains containing the lipid compound globoyltriaosylceramide (Gb3).	globotriaosylceramide	222-225	heat shock protein 1B	Mus musculus	49-55
30413389-4	2018	As previously observed with siRNA knockdown of GLA, globotriaosylceramide (Gb3) accumulated in EA.hy926 cells.	globotriaosylceramide	52-73	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	75-78
31020209-11	2018	Discussion: Fabry disease is a rare X-linked disease caused by mutations on the GLA gene, which leads to low levels of AGAL and accumulation of globotriaosylceramide in the lysosomes of most tissues.	globotriaosylceramide	144-165	galactosidase alpha	Homo sapiens	80-83
30328411-3	2018	alpha-GAL deficient mice (GLA KO) age-dependently accumulate globotriaosylceramide (Gb3) in dorsal root ganglion (DRG) neurons paralleled by endoplasmic stress and apoptosis as contributors to skin denervation.	globotriaosylceramide	61-82	galactosidase, alpha	Mus musculus	0-9
30400144-1	2018	Fabry disease is an X-linked lysosomal storage disease caused by mutations in the GLA gene that lead to a reduction or an absence of the enzyme alpha-galactosidase A, resulting in the progressive and multisystemic accumulation of globotriaosylceramide.	globotriaosylceramide	230-251	galactosidase alpha	Homo sapiens	82-85
30400144-1	2018	Fabry disease is an X-linked lysosomal storage disease caused by mutations in the GLA gene that lead to a reduction or an absence of the enzyme alpha-galactosidase A, resulting in the progressive and multisystemic accumulation of globotriaosylceramide.	globotriaosylceramide	230-251	galactosidase alpha	Homo sapiens	144-165
30481169-3	2018	Here, we carried out Clustered Regularly Interspaced Short Palindromic Repeats (CRISPR)-Cas9-mediated genome-wide loss-of-function screens using two related bacterial toxins, Shiga-like toxins (Stxs) 1 and 2, which use a specific glycolipid, globotriaosylceramide (Gb3), as receptors, and the plant toxin ricin, which recognizes a broad range of glycans.	globotriaosylceramide	242-263	syntaxin 1A	Homo sapiens	175-207
30282881-1	2018	BACKGROUND: Fabry disease is an X-linked lysosomal storage disorder and shows globotriosylceramide (Gb3) accumulation in multiple organs, resulting from a deficiency of alpha-galactosidase.	globotriaosylceramide	78-98	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	100-103
30328411-3	2018	alpha-GAL deficient mice (GLA KO) age-dependently accumulate globotriaosylceramide (Gb3) in dorsal root ganglion (DRG) neurons paralleled by endoplasmic stress and apoptosis as contributors to skin denervation.	globotriaosylceramide	84-87	galactosidase, alpha	Mus musculus	0-9
30328411-7	2018	We show that in vitro alpha-GAL silencing increases intracellular Gb3 accumulation paralleled by loss of Nav1.7 currents, which is reversed by incubation with agalsidase-alpha and lucerastat.	globotriaosylceramide	66-69	galactosidase, alpha	Mus musculus	22-31
29983334-5	2018	Here, we show that primary human monocytes stimulated with Shiga toxin 1a (Stx1a) through the glycolipid receptor globotriaosylceramide released larger amounts of proinflammatory molecules (IL-1beta, TNFalpha, IL-6, G-CSF, CXCL8, CCL2, CCL4) than Stx1a-treated neutrophils.	globotriaosylceramide	114-135	syntaxin 1A	Homo sapiens	75-80
29974530-1	2018	BACKGROUND: The X-linked Fabry disease (FD) is a multiorgan disorder due to alpha-galactosidase A (alpha-GAL) deficiency with consequent lysosomal accumulation of globotriaosylceramide (Gb3).	globotriaosylceramide	163-184	galactosidase alpha	Homo sapiens	99-108
29974530-1	2018	BACKGROUND: The X-linked Fabry disease (FD) is a multiorgan disorder due to alpha-galactosidase A (alpha-GAL) deficiency with consequent lysosomal accumulation of globotriaosylceramide (Gb3).	globotriaosylceramide	163-184	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	186-189
29982630-0	2018	Glucosylceramide synthase inhibition with lucerastat lowers globotriaosylceramide and lysosome staining in cultured fibroblasts from Fabry patients with different mutation types.	globotriaosylceramide	60-81	UDP-glucose ceramide glucosyltransferase	Homo sapiens	0-25
29982630-2	2018	The deleterious mutations lead to accumulation of alpha-GalA substrates, including globotriaosylceramide (Gb3) and globotriaosylsphingosine.	globotriaosylceramide	83-104	galactosidase alpha	Homo sapiens	50-60
29982630-2	2018	The deleterious mutations lead to accumulation of alpha-GalA substrates, including globotriaosylceramide (Gb3) and globotriaosylsphingosine.	globotriaosylceramide	83-104	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	106-109
29927462-2	2018	Reduced alpha-GAL activity leads to progressive accumulation of globotriaosylceramide (Gb3), also known as CD77.	globotriaosylceramide	64-85	galactosidase alpha	Homo sapiens	8-17
29927462-2	2018	Reduced alpha-GAL activity leads to progressive accumulation of globotriaosylceramide (Gb3), also known as CD77.	globotriaosylceramide	64-85	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	87-90
29927462-2	2018	Reduced alpha-GAL activity leads to progressive accumulation of globotriaosylceramide (Gb3), also known as CD77.	globotriaosylceramide	64-85	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	107-111
29983334-5	2018	Here, we show that primary human monocytes stimulated with Shiga toxin 1a (Stx1a) through the glycolipid receptor globotriaosylceramide released larger amounts of proinflammatory molecules (IL-1beta, TNFalpha, IL-6, G-CSF, CXCL8, CCL2, CCL4) than Stx1a-treated neutrophils.	globotriaosylceramide	114-135	interleukin 1 beta	Homo sapiens	190-198
29983334-5	2018	Here, we show that primary human monocytes stimulated with Shiga toxin 1a (Stx1a) through the glycolipid receptor globotriaosylceramide released larger amounts of proinflammatory molecules (IL-1beta, TNFalpha, IL-6, G-CSF, CXCL8, CCL2, CCL4) than Stx1a-treated neutrophils.	globotriaosylceramide	114-135	tumor necrosis factor	Homo sapiens	200-208
29983334-5	2018	Here, we show that primary human monocytes stimulated with Shiga toxin 1a (Stx1a) through the glycolipid receptor globotriaosylceramide released larger amounts of proinflammatory molecules (IL-1beta, TNFalpha, IL-6, G-CSF, CXCL8, CCL2, CCL4) than Stx1a-treated neutrophils.	globotriaosylceramide	114-135	interleukin 6	Homo sapiens	210-214
29983334-5	2018	Here, we show that primary human monocytes stimulated with Shiga toxin 1a (Stx1a) through the glycolipid receptor globotriaosylceramide released larger amounts of proinflammatory molecules (IL-1beta, TNFalpha, IL-6, G-CSF, CXCL8, CCL2, CCL4) than Stx1a-treated neutrophils.	globotriaosylceramide	114-135	colony stimulating factor 3	Homo sapiens	216-221
29983334-5	2018	Here, we show that primary human monocytes stimulated with Shiga toxin 1a (Stx1a) through the glycolipid receptor globotriaosylceramide released larger amounts of proinflammatory molecules (IL-1beta, TNFalpha, IL-6, G-CSF, CXCL8, CCL2, CCL4) than Stx1a-treated neutrophils.	globotriaosylceramide	114-135	C-X-C motif chemokine ligand 8	Homo sapiens	223-228
29983334-5	2018	Here, we show that primary human monocytes stimulated with Shiga toxin 1a (Stx1a) through the glycolipid receptor globotriaosylceramide released larger amounts of proinflammatory molecules (IL-1beta, TNFalpha, IL-6, G-CSF, CXCL8, CCL2, CCL4) than Stx1a-treated neutrophils.	globotriaosylceramide	114-135	C-C motif chemokine ligand 2	Homo sapiens	230-234
29983334-5	2018	Here, we show that primary human monocytes stimulated with Shiga toxin 1a (Stx1a) through the glycolipid receptor globotriaosylceramide released larger amounts of proinflammatory molecules (IL-1beta, TNFalpha, IL-6, G-CSF, CXCL8, CCL2, CCL4) than Stx1a-treated neutrophils.	globotriaosylceramide	114-135	C-C motif chemokine ligand 4	Homo sapiens	236-240
29983334-5	2018	Here, we show that primary human monocytes stimulated with Shiga toxin 1a (Stx1a) through the glycolipid receptor globotriaosylceramide released larger amounts of proinflammatory molecules (IL-1beta, TNFalpha, IL-6, G-CSF, CXCL8, CCL2, CCL4) than Stx1a-treated neutrophils.	globotriaosylceramide	114-135	syntaxin 1A	Homo sapiens	247-252
29866658-3	2018	Globotriaosylceramide and globotetraosylceramide, known as receptors for Stx1a, Stx2a, and Stx2e, and Forssman GSL as a specific receptor for Stx2e, were found to cooccur with SM and cholesterol in DRMs of MDCK II cells, which was shown using TLC overlay assay detection combined with mass spectrometry.	globotriaosylceramide	0-21	STX1A	Canis lupus familiaris	73-78
29549423-6	2018	These cellular effects seem to be mediated by an altered composition of glycosphingolipid-enriched microdomains (GEMs), especially an accumulation of globotriaosylceramide (Gb3) and glucosylceramide (GlcCer), which leads to an activation of Akt and ERK1/2.	globotriaosylceramide	150-171	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	173-176
30211005-2	2018	Fabry disease is a genetic disorder characterized by the accumulation of globotriaosylceramide in cell lysosomes resulting from an X-linked deficiency of alpha-galactosidase A activity.	globotriaosylceramide	73-94	galactosidase alpha	Homo sapiens	154-175
30054149-2	2018	It is caused by deficiency of the enzyme alpha-galactosidase A (alpha-Gal A), which leads to excessive deposition of neutral glycosphingolipids, especially globotriaosylceramide (GL-3), in cells throughout the body.	globotriaosylceramide	156-177	galactosidase, alpha	Mus musculus	41-62
30054149-2	2018	It is caused by deficiency of the enzyme alpha-galactosidase A (alpha-Gal A), which leads to excessive deposition of neutral glycosphingolipids, especially globotriaosylceramide (GL-3), in cells throughout the body.	globotriaosylceramide	156-177	galactosidase, alpha	Mus musculus	64-75
29909504-3	2018	Deficiency in alpha-galactosidase A causes an accumulation of neutral glycosphingolipids such as globotriaosylceramide (Gb3) in lysosomes within various tissues including the vascular endothelium, kidneys, heart, eyes, skin and nervous system.	globotriaosylceramide	97-118	galactosidase alpha	Homo sapiens	14-35
29909504-3	2018	Deficiency in alpha-galactosidase A causes an accumulation of neutral glycosphingolipids such as globotriaosylceramide (Gb3) in lysosomes within various tissues including the vascular endothelium, kidneys, heart, eyes, skin and nervous system.	globotriaosylceramide	97-118	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	120-123
29801874-1	2018	INTRODUCTION: Hypohidrosis and heat intolerance, frequently reported by men and women with Fabry disease (FD), is thought to be related not only to the deposition of globotriaosylceramide (Gb3) in eccrine sweat glands, but also to reduced sweat gland sympathetic innervation.	globotriaosylceramide	166-187	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	189-192
29530250-0	2018	Analysis of globotriaosylceramide (Gb3) isoforms/analogs in unfractionated leukocytes, B lymphocytes and monocytes from Fabry patients using ultra-high performance liquid chromatography/tandem mass spectrometry.	globotriaosylceramide	12-33	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	35-38
29713479-1	2018	Introduction: Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder caused by mutations of GLA gene leading to reduced alpha-galactosidase activity and resulting in a progressive accumulation of globotriaosylceramide (Gb3) and its deacylated derivative, globotriaosyl-sphingosine (Lyso-Gb3).	globotriaosylceramide	212-233	galactosidase alpha	Homo sapiens	108-111
30013462-1	2018	Fabry disease is an X-chromosome linked hereditary disease that is caused by loss of function mutations in the alpha-galactosidase A (alpha-Gal A) gene, resulting in defective glycolipid degradation and subsequent accumulation of globotriaosylceramide (Gb3) in different tissues, including vascular endothelial cells and neurons in the peripheral and central nervous system.	globotriaosylceramide	230-251	galactosidase, alpha	Mus musculus	111-132
30013462-1	2018	Fabry disease is an X-chromosome linked hereditary disease that is caused by loss of function mutations in the alpha-galactosidase A (alpha-Gal A) gene, resulting in defective glycolipid degradation and subsequent accumulation of globotriaosylceramide (Gb3) in different tissues, including vascular endothelial cells and neurons in the peripheral and central nervous system.	globotriaosylceramide	230-251	galactosidase, alpha	Mus musculus	134-145
30013462-1	2018	Fabry disease is an X-chromosome linked hereditary disease that is caused by loss of function mutations in the alpha-galactosidase A (alpha-Gal A) gene, resulting in defective glycolipid degradation and subsequent accumulation of globotriaosylceramide (Gb3) in different tissues, including vascular endothelial cells and neurons in the peripheral and central nervous system.	globotriaosylceramide	253-256	galactosidase, alpha	Mus musculus	111-132
30013462-1	2018	Fabry disease is an X-chromosome linked hereditary disease that is caused by loss of function mutations in the alpha-galactosidase A (alpha-Gal A) gene, resulting in defective glycolipid degradation and subsequent accumulation of globotriaosylceramide (Gb3) in different tissues, including vascular endothelial cells and neurons in the peripheral and central nervous system.	globotriaosylceramide	253-256	galactosidase, alpha	Mus musculus	134-145
29674318-3	2018	Deficiency of human alpha-galactosidase A (alpha-Gal A) causes Fabry disease (FD), a heritable, X-linked lysosomal storage disorder, characterized by accumulation of globotriaosylceramide (Gb3) and globotriaosylsphingosine (lyso-Gb3).	globotriaosylceramide	166-187	galactosidase alpha	Homo sapiens	20-41
29674318-3	2018	Deficiency of human alpha-galactosidase A (alpha-Gal A) causes Fabry disease (FD), a heritable, X-linked lysosomal storage disorder, characterized by accumulation of globotriaosylceramide (Gb3) and globotriaosylsphingosine (lyso-Gb3).	globotriaosylceramide	166-187	galactosidase alpha	Homo sapiens	43-54
29674318-3	2018	Deficiency of human alpha-galactosidase A (alpha-Gal A) causes Fabry disease (FD), a heritable, X-linked lysosomal storage disorder, characterized by accumulation of globotriaosylceramide (Gb3) and globotriaosylsphingosine (lyso-Gb3).	globotriaosylceramide	166-187	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	189-192
29713479-1	2018	Introduction: Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder caused by mutations of GLA gene leading to reduced alpha-galactosidase activity and resulting in a progressive accumulation of globotriaosylceramide (Gb3) and its deacylated derivative, globotriaosyl-sphingosine (Lyso-Gb3).	globotriaosylceramide	212-233	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	235-238
29274327-0	2018	Investigation of correlation of urinary globotriaosylceramide (Gb3) levels with markers of renal function in patients with Fabry disease.	globotriaosylceramide	40-61	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	63-66
29274327-2	2018	The enzymatic defect leads to accumulation of globotriaosylceramide (Gb3) in the kidney.	globotriaosylceramide	46-67	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	69-72
29215092-9	2018	Ten patients received endomyocardial biopsy and all were found to have significant globotriaosylceramide (Gb3) accumulation in their cardiomyocytes.	globotriaosylceramide	83-104	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	106-109
28618999-1	2018	BACKGROUND: Fabry disease is an X-linked lysosomal storage disorder caused by deficient activity of alpha -galactosidase A which leads to progressive intracellular accumulation of globotriaosylceramide in tissues and organs including heart, kidney, vascular endothelium, the nervous system, the eyes and the skin.	globotriaosylceramide	180-201	galactosidase alpha	Homo sapiens	100-122
29582965-1	2018	Background: Fabry disease is a rare genetic lysosomal storage disease, inherited in an X-linked manner, characterized by lysosomal deposition of globotriaosylceramide due to deficient activity of the enzyme alpha-galactosidase A.	globotriaosylceramide	145-166	galactosidase alpha	Homo sapiens	207-228
29558749-2	2018	Complete or partial deficiency in this enzyme leads to intracellular accumulation of globotriaosylceramide (Gb3) and other glycosphingolipids in many cell types throughout the body, including the kidney.	globotriaosylceramide	85-106	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	108-111
28835480-1	2017	BACKGROUND: Fabry disease is characterised by the progressive accumulation of globotriaosylceramide (Gb3) and related glycosphingolipids in vascular endothelial cells.	globotriaosylceramide	78-99	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	101-104
28728877-1	2018	BACKGROUND: Fabry disease (FD) is a rare X-linked lysosomal storage disease caused by mutations in the alpha-galactosidase A (GLA) gene causing deficiency of alpha-galactosidase A which results in progressive glycosphingolipid accumulation, especially globotriaosylceramide (Gb3), in body liquids and lysosomes.	globotriaosylceramide	252-273	galactosidase alpha	Homo sapiens	103-124
28728877-1	2018	BACKGROUND: Fabry disease (FD) is a rare X-linked lysosomal storage disease caused by mutations in the alpha-galactosidase A (GLA) gene causing deficiency of alpha-galactosidase A which results in progressive glycosphingolipid accumulation, especially globotriaosylceramide (Gb3), in body liquids and lysosomes.	globotriaosylceramide	252-273	galactosidase alpha	Homo sapiens	126-129
28728877-1	2018	BACKGROUND: Fabry disease (FD) is a rare X-linked lysosomal storage disease caused by mutations in the alpha-galactosidase A (GLA) gene causing deficiency of alpha-galactosidase A which results in progressive glycosphingolipid accumulation, especially globotriaosylceramide (Gb3), in body liquids and lysosomes.	globotriaosylceramide	252-273	galactosidase alpha	Homo sapiens	158-179
29099167-2	2017	This enzyme contributes to the cellular recycling of glycosphingolipids such as galabiosylceramide (Ga2), globotriaosylceramide (Gb3), and globotriaosylsphingosine (lyso-Gb3) by hydrolyzing the terminal alpha-galactosyl moiety.	globotriaosylceramide	106-127	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	129-132
28756410-1	2017	OBJECTIVE: Deficiency of alpha-galactosidase A (alphaGal-A) in Fabry disease leads to the accumulation mainly of globotriaosylceramide (GL3) in multiple renal cell types.	globotriaosylceramide	113-134	galactosidase, alpha	Mus musculus	25-46
28674962-1	2017	Fabry disease resulting from a deficiency of alpha-galactosidase A leads to the accumulation of globotriaosylceramide in various organs.	globotriaosylceramide	96-117	galactosidase alpha	Homo sapiens	45-66
28593486-1	2017	Fabry disease is an X-linked lysosomal storage disorder caused by a lack of alpha-galactosidase A activity, which leads to the accumulation of globotriaosylceramide in various organs.	globotriaosylceramide	143-164	galactosidase alpha	Homo sapiens	76-97
28877708-1	2017	BACKGROUND: Fabry disease is an X-linked lysosomal storage disorder caused by deficient activity of alpha-galactosidase A and the resulting accumulation of the glycosphingolipid globotriaosylceramide (Gb3) and its derivatives, including globotriaosylsphingosine (Lyso-Gb3).	globotriaosylceramide	178-199	galactosidase alpha	Homo sapiens	100-121
29068380-3	2017	Only limited data are available regarding precise structures of their Stx receptor glycosphingolipids (GSLs) globotriaosylceramide (Gb3Cer) and globotetraosylceramide (Gb4Cer), and lipid raft association.	globotriaosylceramide	109-130	ST8 alpha-N-acetyl-neuraminide alpha-2,8-sialyltransferase 2	Homo sapiens	70-73
29068380-3	2017	Only limited data are available regarding precise structures of their Stx receptor glycosphingolipids (GSLs) globotriaosylceramide (Gb3Cer) and globotetraosylceramide (Gb4Cer), and lipid raft association.	globotriaosylceramide	132-138	ST8 alpha-N-acetyl-neuraminide alpha-2,8-sialyltransferase 2	Homo sapiens	70-73
28947349-7	2017	Innate immunity is activated by signals originating from dendritic cells via interactions between toll-like receptors and globotriaosylceramide (Gb3) and/or globotriaosylsphingosine (lyso-Gb3).	globotriaosylceramide	122-143	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	145-148
28535204-6	2017	Exposure of primary HRGECs to the ceramide analogon d-threo-1-phenyl-2-decanoylamino-3-morpholino-1-propanol (D-PDMP) reduced total Gb3Cer and Gb4Cer content, roughly calculated from two biological replicates, down to half and quarter of its primordial content, respectively, but strengthened their prevalence and cholesterol preponderance in DRMs.	globotriaosylceramide	132-138	beta-1,3-N-acetylgalactosaminyltransferase 1 (globoside blood group)	Homo sapiens	143-149
28625968-11	2017	Podocyte globotriaosylceramide (Gb3) reduction correlated with cumulative agalsidase dose (r=0.69; P=0.001).	globotriaosylceramide	9-30	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	32-35
28877708-1	2017	BACKGROUND: Fabry disease is an X-linked lysosomal storage disorder caused by deficient activity of alpha-galactosidase A and the resulting accumulation of the glycosphingolipid globotriaosylceramide (Gb3) and its derivatives, including globotriaosylsphingosine (Lyso-Gb3).	globotriaosylceramide	178-199	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	201-204
28877708-1	2017	BACKGROUND: Fabry disease is an X-linked lysosomal storage disorder caused by deficient activity of alpha-galactosidase A and the resulting accumulation of the glycosphingolipid globotriaosylceramide (Gb3) and its derivatives, including globotriaosylsphingosine (Lyso-Gb3).	globotriaosylceramide	178-199	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	268-271
28847675-1	2017	Fabry disease (FD) results from impaired globotriaosylceramide (Gb3) catabolism, due to a deficiency of the lysosomal hydrolase, alpha-galactosidase A (alpha-GalA).	globotriaosylceramide	41-62	galactosidase alpha	Homo sapiens	129-150
28847675-1	2017	Fabry disease (FD) results from impaired globotriaosylceramide (Gb3) catabolism, due to a deficiency of the lysosomal hydrolase, alpha-galactosidase A (alpha-GalA).	globotriaosylceramide	41-62	galactosidase alpha	Homo sapiens	152-162
28847675-1	2017	Fabry disease (FD) results from impaired globotriaosylceramide (Gb3) catabolism, due to a deficiency of the lysosomal hydrolase, alpha-galactosidase A (alpha-GalA).	globotriaosylceramide	64-67	galactosidase alpha	Homo sapiens	129-150
28847675-1	2017	Fabry disease (FD) results from impaired globotriaosylceramide (Gb3) catabolism, due to a deficiency of the lysosomal hydrolase, alpha-galactosidase A (alpha-GalA).	globotriaosylceramide	64-67	galactosidase alpha	Homo sapiens	152-162
28662189-1	2017	Fabry disease is an X-linked inherited lysosomal storage disorder with intracellular accumulation of globotriaosylceramide (Gb3) due to alpha-galactosidase A (alpha-Gal A) deficiency.	globotriaosylceramide	101-122	galactosidase, alpha	Mus musculus	136-157
28663131-1	2017	BACKGROUND: Fabry disease (FD), an X-linked lysosomal storage disorder, results from the deficient activity of alpha-galactosidase A (alpha-Gal A) and the accumulation of its substrates, globotriaosylceramide (Gb3) and its deacylated derivative, globotriaosyl-sphingosine (Lyso-Gb3).	globotriaosylceramide	187-208	galactosidase alpha	Homo sapiens	111-132
28663131-1	2017	BACKGROUND: Fabry disease (FD), an X-linked lysosomal storage disorder, results from the deficient activity of alpha-galactosidase A (alpha-Gal A) and the accumulation of its substrates, globotriaosylceramide (Gb3) and its deacylated derivative, globotriaosyl-sphingosine (Lyso-Gb3).	globotriaosylceramide	187-208	galactosidase alpha	Homo sapiens	134-145
28662189-1	2017	Fabry disease is an X-linked inherited lysosomal storage disorder with intracellular accumulation of globotriaosylceramide (Gb3) due to alpha-galactosidase A (alpha-Gal A) deficiency.	globotriaosylceramide	101-122	galactosidase, alpha	Mus musculus	159-170
28161408-2	2017	Fabry disease (FD) is caused by a deficiency of alpha-galactosidase (GLA), which results in the accumulation of globotriaosylceramide (GL-3).	globotriaosylceramide	112-133	galactosidase, alpha	Mus musculus	69-72
28702361-1	2017	Fabry disease (FD) [OMIM 301500] is an X-linked lysosomal storage disorder caused by a deficiency of the lysosomal enzyme alpha-galactosidase A, resulting in progressive multisystem accumulation of globotriaosylceramide (Gb3).	globotriaosylceramide	198-219	galactosidase alpha	Homo sapiens	122-143
27938475-2	2017	Progressive intracellular accumulation of globotriaosylceramide (Gb3) is considered to be pathogenically responsible for the phenotype variability of FD that causes cardiovascular dysfunction; however, molecular mechanisms underlying the impairment of FD-associated cardiovascular tissues remain unclear.	globotriaosylceramide	42-63	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	65-68
28384397-0	2017	High-Risk Screening for Fabry Disease: Analysis by Tandem Mass Spectrometry of Globotriaosylceramide (Gb3 ) in Urine Collected on Filter Paper.	globotriaosylceramide	79-100	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	102-105
28384397-7	2017	The purpose of this protocol is to focus on the high-risk screening of patients who might have Fabry disease using a simple, rapid, non-invasive high performance liquid chromatography-tandem mass spectrometry (HPLC-MS/MS) method for urinary globotriaosylceramide (Gb3 ) analysis.	globotriaosylceramide	241-262	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	264-267
28165371-1	2017	Shiga toxins consist of an A-moiety and five B-moieties able to bind the neutral glycosphingolipid globotriaosylceramide (Gb3) on the cell surface.	globotriaosylceramide	99-120	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	122-125
27943675-1	2017	The bacterial Shiga toxin interacts with its cellular receptor, the glycosphingolipid globotriaosylceramide (Gb3 or CD77), as a first step to entering target cells.	globotriaosylceramide	86-107	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	109-112
27943675-1	2017	The bacterial Shiga toxin interacts with its cellular receptor, the glycosphingolipid globotriaosylceramide (Gb3 or CD77), as a first step to entering target cells.	globotriaosylceramide	86-107	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	116-120
28075357-1	2017	We retrospectively evaluated correlations between cardiac manifestations and globotriaosylceramide (Gb3) accumulation in cardiomyocytes from Taiwanese patients with Fabry disease and the IVS4+919G>A (IVS4) mutation who underwent endomyocardial biopsy (Shire; Fabry Outcome Survey data; extracted January 2015).	globotriaosylceramide	77-98	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	100-103
28601129-2	2017	Consequently, there is very low, or even absent, activity of the lysosomal enzyme alpha-galactosidase A (alpha-Gal A), resulting in the progressive accumulation of glycosphingolipids (predominantly, globotriaosylceramide (GL-3)) in various cells of different organs.	globotriaosylceramide	199-220	galactosidase, alpha	Mus musculus	82-103
28049500-2	2017	Clinical onset of Fabry disease is preceded by significant storage of globotriaosylceramide (Gb3) and related glycosphingolipids, but the extent of the metabolic progression before symptoms is unknown.	globotriaosylceramide	70-91	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	93-96
28601129-2	2017	Consequently, there is very low, or even absent, activity of the lysosomal enzyme alpha-galactosidase A (alpha-Gal A), resulting in the progressive accumulation of glycosphingolipids (predominantly, globotriaosylceramide (GL-3)) in various cells of different organs.	globotriaosylceramide	199-220	galactosidase, alpha	Mus musculus	105-116
27558838-2	2017	Stxs are AB5 toxins and the B-pentamers of the two major Stx subtypes Stx1a and Stx2a preferentially bind to the glycosphingolipid (GSL) globotriaosylceramide (Gb3Cer) expressed by human endothelial cells.	globotriaosylceramide	137-158	syntaxin 1A	Homo sapiens	70-75
27558838-2	2017	Stxs are AB5 toxins and the B-pentamers of the two major Stx subtypes Stx1a and Stx2a preferentially bind to the glycosphingolipid (GSL) globotriaosylceramide (Gb3Cer) expressed by human endothelial cells.	globotriaosylceramide	137-158	syntaxin 2	Homo sapiens	80-85
27558838-2	2017	Stxs are AB5 toxins and the B-pentamers of the two major Stx subtypes Stx1a and Stx2a preferentially bind to the glycosphingolipid (GSL) globotriaosylceramide (Gb3Cer) expressed by human endothelial cells.	globotriaosylceramide	160-166	syntaxin 1A	Homo sapiens	70-75
27558838-2	2017	Stxs are AB5 toxins and the B-pentamers of the two major Stx subtypes Stx1a and Stx2a preferentially bind to the glycosphingolipid (GSL) globotriaosylceramide (Gb3Cer) expressed by human endothelial cells.	globotriaosylceramide	160-166	syntaxin 2	Homo sapiens	80-85
27458128-2	2016	Its substrates, mainly globotriaosylceramide (Gb3), accumulate and seem to induce other pathophysiological findings of FD.	globotriaosylceramide	23-44	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	46-49
29098097-1	2017	Fabry disease is an X-linked lysosomal storage disorder resulting from the deficiency or absence of the enzyme alpha galactosidase A; this defect leads to the systemic accumulation of globotriaosylceramide and its metabolites.	globotriaosylceramide	184-205	galactosidase alpha	Homo sapiens	111-132
27756537-1	2017	In Fabry disease, large amounts of globotriaosylceramide (Gb3) and related glycosphingolipids accumulate in organs due to a deficiency of alpha-galactosidase A (GLA) activity.	globotriaosylceramide	35-56	galactosidase, alpha	Mus musculus	138-159
27756537-1	2017	In Fabry disease, large amounts of globotriaosylceramide (Gb3) and related glycosphingolipids accumulate in organs due to a deficiency of alpha-galactosidase A (GLA) activity.	globotriaosylceramide	35-56	galactosidase, alpha	Mus musculus	161-164
27773586-2	2017	As a result, the major glycosphingolipid substrates, globotriaosylceramide (Gb3) and globotriaosylsphingosine (LysoGb3) accumulate in plasma, urine and tissue lysosomes.	globotriaosylceramide	53-74	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	76-79
28933415-1	2016	Fabry disease (FD) is a rare X-linked recessive genetic disorder caused by a deficient activity of the lysosomal enzyme alpha-galactosidase A (GLA) and is characterised by intra-lysosomal accumulation of globotriaosylceramide (Gb3).	globotriaosylceramide	204-225	galactosidase alpha	Homo sapiens	120-141
28933415-1	2016	Fabry disease (FD) is a rare X-linked recessive genetic disorder caused by a deficient activity of the lysosomal enzyme alpha-galactosidase A (GLA) and is characterised by intra-lysosomal accumulation of globotriaosylceramide (Gb3).	globotriaosylceramide	204-225	galactosidase alpha	Homo sapiens	143-146
27195818-4	2016	Here, we review the various types of GLA variants and recommend that pathogenicity be considered only when associated with elevated globotriaosylceramide in disease-relevant organs and tissues as analyzed by mass spectrometry.	globotriaosylceramide	132-153	galactosidase alpha	Homo sapiens	37-40
27112153-2	2016	Human alpha-galactosidase A (halphaGAL) hydrolyses the terminal alpha-galactosyl moiety from glycosphingolipids, predominantly globotriaosylceramide (Gb3).	globotriaosylceramide	127-148	galactosidase alpha	Homo sapiens	6-27
27225543-1	2016	OPINION STATEMENT: Fabry disease is an X-linked, lysosomal storage disorder caused by a mutation in the GLA gene leading to a deficiency in alpha-galactosidase A enzyme (alpha-Gal A) activity, which in turn results in accumulation of globotriaosylceramide in the vascular endothelium and smooth muscle cells of different organs, including kidney and heart, finally leading to impairment or failure of organ function.	globotriaosylceramide	234-255	galactosidase alpha	Homo sapiens	104-107
27083555-1	2016	BACKGROUND: Fabry disease (FD) is an X-linked lysosomal storage disorder caused by a deficiency of alpha-galactosidase A enzyme, which leads to the accumulation of its substrate, the globotriaosylceramide or Gb3, in many organs and tissues.	globotriaosylceramide	183-204	galactosidase alpha	Homo sapiens	99-120
27070906-2	2016	Upon STxB-mediated binding to the glycolipid globotriaosylceramide (Gb3) at the plasma membrane of target cells, Shiga toxin is internalized by clathrin-dependent and independent endocytosis.	globotriaosylceramide	45-66	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	68-71
28649509-1	2017	Fabry disease (FD) is an X-linked lysosomal storage disorder caused by loss of function mutations in the GLA gene at Xq22 with subsequent functional deficiency of alpha-galactosidase A, resulting in the accumulation of globotriaosylceramide (GL-3 or Gb3) in multiple cells types throughout the body.	globotriaosylceramide	219-240	galactosidase, alpha	Mus musculus	105-108
28649509-1	2017	Fabry disease (FD) is an X-linked lysosomal storage disorder caused by loss of function mutations in the GLA gene at Xq22 with subsequent functional deficiency of alpha-galactosidase A, resulting in the accumulation of globotriaosylceramide (GL-3 or Gb3) in multiple cells types throughout the body.	globotriaosylceramide	219-240	galactosidase, alpha	Mus musculus	163-184
27531673-2	2016	alpha-GalA deficiency leads to multisystemic clinical manifestations caused by the preferential accumulation of globotriaosylceramide (Gb3) in the endothelium and vascular smooth muscles.	globotriaosylceramide	112-133	galactosidase, alpha	Mus musculus	0-10
27225543-1	2016	OPINION STATEMENT: Fabry disease is an X-linked, lysosomal storage disorder caused by a mutation in the GLA gene leading to a deficiency in alpha-galactosidase A enzyme (alpha-Gal A) activity, which in turn results in accumulation of globotriaosylceramide in the vascular endothelium and smooth muscle cells of different organs, including kidney and heart, finally leading to impairment or failure of organ function.	globotriaosylceramide	234-255	galactosidase alpha	Homo sapiens	140-168
27225543-1	2016	OPINION STATEMENT: Fabry disease is an X-linked, lysosomal storage disorder caused by a mutation in the GLA gene leading to a deficiency in alpha-galactosidase A enzyme (alpha-Gal A) activity, which in turn results in accumulation of globotriaosylceramide in the vascular endothelium and smooth muscle cells of different organs, including kidney and heart, finally leading to impairment or failure of organ function.	globotriaosylceramide	234-255	galactosidase alpha	Homo sapiens	170-181
27367162-2	2016	Currently, globotriaosylsphingosine (lyso-Gb3 ) and globotriaosylceramide (Gb3 ) are used as biomarkers to diagnose and monitor Fabry patients.	globotriaosylceramide	52-73	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	75-78
27367163-1	2016	Fabry disease is an X-linked lysosomal storage disorder, caused by a deficit in alpha-galactosidase A enzyme activity, leading to the storage of sphingolipids such as globotriaosylsphingosine (lyso-Gb3 ), globotriaosylceramide (Gb3 ), and galabiosylceramide (Ga2 ) in organs, tissues and biological fluids.	globotriaosylceramide	205-226	galactosidase alpha	Homo sapiens	80-101
27438980-1	2016	Every cell in the human body has globotriaosylceramide accumulation (Gb3) in Fabry disease due to the mutation in gene of the enzyme alpha-galactosidase A.	globotriaosylceramide	33-54	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	69-72
27438980-1	2016	Every cell in the human body has globotriaosylceramide accumulation (Gb3) in Fabry disease due to the mutation in gene of the enzyme alpha-galactosidase A.	globotriaosylceramide	33-54	galactosidase alpha	Homo sapiens	133-154
27112153-2	2016	Human alpha-galactosidase A (halphaGAL) hydrolyses the terminal alpha-galactosyl moiety from glycosphingolipids, predominantly globotriaosylceramide (Gb3).	globotriaosylceramide	127-148	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	150-153
27398254-1	2016	Fabry"s disease is an X-linked lysosomal storage disorder caused by a deficiency of alpha-galactosidase A enzyme with the progressive accumulation of globotriaosylceramide in vascular endothelial cells leading to cardiovascular, renal, gastrointestinal, neuropathic, lenticular, and dermatological manifestations.	globotriaosylceramide	150-171	galactosidase alpha	Homo sapiens	84-105
27145802-1	2016	BACKGROUND: Fabry disease is an X-linked lysosomal storage disorder due to impaired activity of alpha-galactosidase A with intracellular accumulation of globotriaosylceramide.	globotriaosylceramide	153-174	galactosidase, alpha	Mus musculus	96-117
26470913-2	2016	This study investigated the localization of globotriaosylceramide (Gb3) in human brain and kidney tissues removed from forensic autopsy cases in Japan.	globotriaosylceramide	44-65	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	67-70
27061865-1	2016	Fabry disease is an inherited, X-linked lysosomal storage disorder caused by deficiency of the enzyme alpha galactosidase A (alpha-GLA A), which leads to glycosphingolipid accumulation, mainly globotriaosylceramide, in tissues.	globotriaosylceramide	193-214	galactosidase alpha	Homo sapiens	102-123
27061865-1	2016	Fabry disease is an inherited, X-linked lysosomal storage disorder caused by deficiency of the enzyme alpha galactosidase A (alpha-GLA A), which leads to glycosphingolipid accumulation, mainly globotriaosylceramide, in tissues.	globotriaosylceramide	193-214	galactosidase alpha	Homo sapiens	125-136
26826119-2	2016	Here, we tested whether human gastric cancers, which are among the most aggressive tumor entities, express the cellular receptor of Shiga toxin, the glycosphingolipid globotriaosylceramide (Gb3/CD77).	globotriaosylceramide	167-188	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	190-193
26826119-2	2016	Here, we tested whether human gastric cancers, which are among the most aggressive tumor entities, express the cellular receptor of Shiga toxin, the glycosphingolipid globotriaosylceramide (Gb3/CD77).	globotriaosylceramide	167-188	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	194-198
27123349-2	2016	It is characterized by progressive lysosomal accumulation of globotriaosylceramide (Gb3) and multisystem pathology, affecting the skin, nervous and cerebrovascular systems, kidneys, and heart.	globotriaosylceramide	61-82	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	84-87
26773500-1	2016	Human Gb3/CD77 synthase (alpha1,4-galactosyltransferase, P(k) synthase), encoded by A4GALT gene, is known for synthesis of Gal(alpha1-4)Gal moiety in globotriaosylceramide (Gb3Cer, CD77, P(k) blood group antigen), a glycosphingolipid of the globo series.	globotriaosylceramide	150-171	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	6-9
26773500-1	2016	Human Gb3/CD77 synthase (alpha1,4-galactosyltransferase, P(k) synthase), encoded by A4GALT gene, is known for synthesis of Gal(alpha1-4)Gal moiety in globotriaosylceramide (Gb3Cer, CD77, P(k) blood group antigen), a glycosphingolipid of the globo series.	globotriaosylceramide	150-171	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	10-23
26773500-1	2016	Human Gb3/CD77 synthase (alpha1,4-galactosyltransferase, P(k) synthase), encoded by A4GALT gene, is known for synthesis of Gal(alpha1-4)Gal moiety in globotriaosylceramide (Gb3Cer, CD77, P(k) blood group antigen), a glycosphingolipid of the globo series.	globotriaosylceramide	150-171	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	25-55
26773500-1	2016	Human Gb3/CD77 synthase (alpha1,4-galactosyltransferase, P(k) synthase), encoded by A4GALT gene, is known for synthesis of Gal(alpha1-4)Gal moiety in globotriaosylceramide (Gb3Cer, CD77, P(k) blood group antigen), a glycosphingolipid of the globo series.	globotriaosylceramide	150-171	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	84-90
26773500-1	2016	Human Gb3/CD77 synthase (alpha1,4-galactosyltransferase, P(k) synthase), encoded by A4GALT gene, is known for synthesis of Gal(alpha1-4)Gal moiety in globotriaosylceramide (Gb3Cer, CD77, P(k) blood group antigen), a glycosphingolipid of the globo series.	globotriaosylceramide	150-171	adrenoceptor alpha 1D	Homo sapiens	127-135
26773500-1	2016	Human Gb3/CD77 synthase (alpha1,4-galactosyltransferase, P(k) synthase), encoded by A4GALT gene, is known for synthesis of Gal(alpha1-4)Gal moiety in globotriaosylceramide (Gb3Cer, CD77, P(k) blood group antigen), a glycosphingolipid of the globo series.	globotriaosylceramide	150-171	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	10-14
26773500-1	2016	Human Gb3/CD77 synthase (alpha1,4-galactosyltransferase, P(k) synthase), encoded by A4GALT gene, is known for synthesis of Gal(alpha1-4)Gal moiety in globotriaosylceramide (Gb3Cer, CD77, P(k) blood group antigen), a glycosphingolipid of the globo series.	globotriaosylceramide	173-179	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	6-9
26773500-1	2016	Human Gb3/CD77 synthase (alpha1,4-galactosyltransferase, P(k) synthase), encoded by A4GALT gene, is known for synthesis of Gal(alpha1-4)Gal moiety in globotriaosylceramide (Gb3Cer, CD77, P(k) blood group antigen), a glycosphingolipid of the globo series.	globotriaosylceramide	173-179	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	10-23
26773500-1	2016	Human Gb3/CD77 synthase (alpha1,4-galactosyltransferase, P(k) synthase), encoded by A4GALT gene, is known for synthesis of Gal(alpha1-4)Gal moiety in globotriaosylceramide (Gb3Cer, CD77, P(k) blood group antigen), a glycosphingolipid of the globo series.	globotriaosylceramide	173-179	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	25-55
26773500-1	2016	Human Gb3/CD77 synthase (alpha1,4-galactosyltransferase, P(k) synthase), encoded by A4GALT gene, is known for synthesis of Gal(alpha1-4)Gal moiety in globotriaosylceramide (Gb3Cer, CD77, P(k) blood group antigen), a glycosphingolipid of the globo series.	globotriaosylceramide	173-179	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	84-90
26773500-1	2016	Human Gb3/CD77 synthase (alpha1,4-galactosyltransferase, P(k) synthase), encoded by A4GALT gene, is known for synthesis of Gal(alpha1-4)Gal moiety in globotriaosylceramide (Gb3Cer, CD77, P(k) blood group antigen), a glycosphingolipid of the globo series.	globotriaosylceramide	173-179	adrenoceptor alpha 1D	Homo sapiens	127-135
26773500-1	2016	Human Gb3/CD77 synthase (alpha1,4-galactosyltransferase, P(k) synthase), encoded by A4GALT gene, is known for synthesis of Gal(alpha1-4)Gal moiety in globotriaosylceramide (Gb3Cer, CD77, P(k) blood group antigen), a glycosphingolipid of the globo series.	globotriaosylceramide	173-179	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	10-14
26593248-2	2016	Globotriaosylceramide (Gb3) and globotriaosylsphingosine (lyso-Gb3) are currently used for Fabry screening and diagnosis.	globotriaosylceramide	0-21	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	23-26
26797827-2	2016	Globotriaosylceramide (Gb3) and its isoforms and analogues have been identified and quantified as biomarkers of disease severity and treatment efficacy.	globotriaosylceramide	0-21	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	23-26
27047943-1	2016	Fabry disease is an X-chromosome-linked lysosomal storage disease characterized by a deficient activity or, in most males, absence of the enzyme alpha-galactosidase A (a-Gal A) leading to systemic, primary lysosomal accumulation of globotriaosylceramide (Gb3) (1).	globotriaosylceramide	232-253	galactosidase alpha	Homo sapiens	145-166
27047943-1	2016	Fabry disease is an X-chromosome-linked lysosomal storage disease characterized by a deficient activity or, in most males, absence of the enzyme alpha-galactosidase A (a-Gal A) leading to systemic, primary lysosomal accumulation of globotriaosylceramide (Gb3) (1).	globotriaosylceramide	232-253	galactosidase alpha	Homo sapiens	168-175
26454753-1	2016	UNLABELLED: Fabry disease is an X-linked glycosphingolipidosis caused by deficient synthesis of the enzyme alpha-galactosidase A, which results in accumulations of globotriaosylceramide (GL-3) in systemic tissues.	globotriaosylceramide	164-185	galactosidase alpha	Homo sapiens	107-128
26928672-5	2016	Using Annexin V (AV) and Shiga toxin B subunit (ST) with affinities for phosphatidylserine and globotriaosylceramide, respectively, AV- and a ST-binding EV were identified.	globotriaosylceramide	95-116	annexin A5	Homo sapiens	6-15
26683465-0	2016	A Short Synthetic Peptide Mimetic of Apolipoprotein A1 Mediates Cholesterol and Globotriaosylceramide Efflux from Fabry Fibroblasts.	globotriaosylceramide	80-101	apolipoprotein A1	Homo sapiens	37-54
26333625-2	2016	Fabry disease is an X-linked metabolic storage disorder due to the deficiency of lysosomal alpha-galactosidase A which causes accumulation of glycosphingolipids, primarily globotriaosylceramide, throughout the body.	globotriaosylceramide	172-193	galactosidase alpha	Homo sapiens	91-112
26426881-0	2016	Globotriaosylceramide inhibits iNKT-cell activation in a CD1d-dependent manner.	globotriaosylceramide	0-21	CD1d molecule	Homo sapiens	57-61
26426881-1	2016	Globotriaosylceramide (Gb3) is a glycosphingolipid present in cellular membranes that progressively accumulates in Fabry disease.	globotriaosylceramide	0-21	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	23-26
26683465-1	2016	Fabry disease is an X-linked sphingolipid storage disorder caused by a deficiency of the lysosomal enzyme alpha-galactosidase A (AGA, EC 3.2.1.22) resulting in the intracellular accumulation of globotriaosylceramide (Gb3).	globotriaosylceramide	194-215	galactosidase alpha	Homo sapiens	106-127
26683465-1	2016	Fabry disease is an X-linked sphingolipid storage disorder caused by a deficiency of the lysosomal enzyme alpha-galactosidase A (AGA, EC 3.2.1.22) resulting in the intracellular accumulation of globotriaosylceramide (Gb3).	globotriaosylceramide	194-215	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	217-220
25857295-0	2015	Interfering parameters in the determination of urinary globotriaosylceramide (Gb3) in patients with chronic kidney disease.	globotriaosylceramide	55-76	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	78-81
26835405-2	2016	Mutations of the GLA gene result in deficiency of the lysosomal enzyme, alpha-galactosidase A (alpha-Gal A) with accumulation of glycosphingolipids, particularly globotriaosylceramide (GL3) in the vascular endothelium of various tissues.	globotriaosylceramide	162-183	galactosidase alpha	Homo sapiens	17-20
26835405-2	2016	Mutations of the GLA gene result in deficiency of the lysosomal enzyme, alpha-galactosidase A (alpha-Gal A) with accumulation of glycosphingolipids, particularly globotriaosylceramide (GL3) in the vascular endothelium of various tissues.	globotriaosylceramide	162-183	galactosidase alpha	Homo sapiens	95-106
27735906-2	2016	Fabry disease (Anderson-Fabry disease) is an X-linked recessive lysosomal storage disorder resulting from deficient activity of lysosomal hydrolase, alpha-galactosidase A (alpha-Gal A), which leads to progressive accumulation of globotriaosylceramide (Gb3) in various cells, predominantly endothelial and vascular smooth muscle cells, with clinical manifestations affecting major organs including the central nervous system.	globotriaosylceramide	229-250	galactosidase alpha	Homo sapiens	149-170
27735906-2	2016	Fabry disease (Anderson-Fabry disease) is an X-linked recessive lysosomal storage disorder resulting from deficient activity of lysosomal hydrolase, alpha-galactosidase A (alpha-Gal A), which leads to progressive accumulation of globotriaosylceramide (Gb3) in various cells, predominantly endothelial and vascular smooth muscle cells, with clinical manifestations affecting major organs including the central nervous system.	globotriaosylceramide	229-250	galactosidase alpha	Homo sapiens	172-183
26592662-2	2015	The disease ultimately manifests as multiple organ dysfunctions owing to excessive accumulation of globotriaosylceramide (Gb3).	globotriaosylceramide	99-120	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	122-125
25857295-1	2015	INTRODUCTION: Globotriaosylceramide (Gb3, CD77) represents a pivotal part of the cell membrane.	globotriaosylceramide	14-35	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	37-40
25857295-1	2015	INTRODUCTION: Globotriaosylceramide (Gb3, CD77) represents a pivotal part of the cell membrane.	globotriaosylceramide	14-35	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	42-46
25697597-2	2015	Globotriaosylsphingosine (lyso-Gb3), a deacylated form of globotriaosylceramide (Gb3), has emerged as a marker of Fabry disease.	globotriaosylceramide	58-79	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	31-34
26464281-5	2015	We identified globotriaosylceramide (Gb3Cer) and globotetraosylceramide (Gb4Cer) as the GSL receptors for Stx1a, Stx2a, and Stx2e subtypes using TLC overlay detection combined with MS.	globotriaosylceramide	14-35	syntaxin-1A	Chlorocebus sabaeus	106-111
26464281-5	2015	We identified globotriaosylceramide (Gb3Cer) and globotetraosylceramide (Gb4Cer) as the GSL receptors for Stx1a, Stx2a, and Stx2e subtypes using TLC overlay detection combined with MS.	globotriaosylceramide	14-35	syntaxin-2	Chlorocebus sabaeus	113-117
26464281-5	2015	We identified globotriaosylceramide (Gb3Cer) and globotetraosylceramide (Gb4Cer) as the GSL receptors for Stx1a, Stx2a, and Stx2e subtypes using TLC overlay detection combined with MS.	globotriaosylceramide	37-43	syntaxin-1A	Chlorocebus sabaeus	106-111
26502906-4	2016	The treatment of cells with a chemical inhibitor of glycosphingolipid biosynthesis, which suppresses the expression of the Stx receptor globotriaosylceramide and subsequent endocytosis of the toxin, substantially blocked activation of the NLRP3 inflammasome and processing of caspase-1 and IL-1beta.	globotriaosylceramide	136-157	NLR family pyrin domain containing 3	Homo sapiens	239-244
26055721-2	2015	Globoside or P antigen is synthesized by UDP-N-acetylgalactosamine:globotriaosyl-ceramide 3-beta-N-acetylgalactosaminyltransferase encoded by B3GALNT1.	globotriaosylceramide	67-89	beta-1,3-N-acetylgalactosaminyltransferase 1 (globoside blood group)	Homo sapiens	142-150
26070511-0	2015	Variations in the GLA gene correlate with globotriaosylceramide and globotriaosylsphingosine analog levels in urine and plasma.	globotriaosylceramide	42-63	galactosidase alpha	Homo sapiens	18-21
26070511-1	2015	Recent data have shown that lyso-Gb3, the deacylated derivative of globotriaosylceramide (Gb3), is possibly involved in the pathogenesis of Fabry disease (FD) and might be a clinically useful biomarker of its metabolic load.	globotriaosylceramide	67-88	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	33-36
26070511-1	2015	Recent data have shown that lyso-Gb3, the deacylated derivative of globotriaosylceramide (Gb3), is possibly involved in the pathogenesis of Fabry disease (FD) and might be a clinically useful biomarker of its metabolic load.	globotriaosylceramide	67-88	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	90-93
25666440-1	2015	Fabry disease (FD) is an X-linked lysosomal storage disorder caused by a deficiency of the lysosomal hydrolase alpha-galactosidase A (alpha-GalA) that leads to the intra-lysosomal accumulation of globotriaosylceramide (Gb3) in various organ systems.	globotriaosylceramide	196-217	galactosidase alpha	Homo sapiens	111-132
25666440-1	2015	Fabry disease (FD) is an X-linked lysosomal storage disorder caused by a deficiency of the lysosomal hydrolase alpha-galactosidase A (alpha-GalA) that leads to the intra-lysosomal accumulation of globotriaosylceramide (Gb3) in various organ systems.	globotriaosylceramide	196-217	galactosidase alpha	Homo sapiens	134-144
26464281-5	2015	We identified globotriaosylceramide (Gb3Cer) and globotetraosylceramide (Gb4Cer) as the GSL receptors for Stx1a, Stx2a, and Stx2e subtypes using TLC overlay detection combined with MS.	globotriaosylceramide	37-43	syntaxin-2	Chlorocebus sabaeus	113-117
26135632-1	2015	Fabry disease is an X-linked lysosomal storage disorder caused by mutations in the gene encoding the alpha-galactosidase A (alpha-Gal A) lysosomal enzyme, which results in globotriaosylceramide (Gb3) storage in vascular endothelial cells and different cell types throughout the body.	globotriaosylceramide	172-193	galactosidase alpha	Homo sapiens	101-122
26135632-1	2015	Fabry disease is an X-linked lysosomal storage disorder caused by mutations in the gene encoding the alpha-galactosidase A (alpha-Gal A) lysosomal enzyme, which results in globotriaosylceramide (Gb3) storage in vascular endothelial cells and different cell types throughout the body.	globotriaosylceramide	172-193	galactosidase alpha	Homo sapiens	124-135
26135632-1	2015	Fabry disease is an X-linked lysosomal storage disorder caused by mutations in the gene encoding the alpha-galactosidase A (alpha-Gal A) lysosomal enzyme, which results in globotriaosylceramide (Gb3) storage in vascular endothelial cells and different cell types throughout the body.	globotriaosylceramide	172-193	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	195-198
26291612-1	2015	Fabry disease is a lysosomal storage disorder caused by deficiency of alpha-galactosidase A (alpha-gal A), which results in the deposition of globotriaosylceramide (Gb3) in the vascular endothelium.	globotriaosylceramide	142-163	galactosidase alpha	Homo sapiens	70-91
26291612-1	2015	Fabry disease is a lysosomal storage disorder caused by deficiency of alpha-galactosidase A (alpha-gal A), which results in the deposition of globotriaosylceramide (Gb3) in the vascular endothelium.	globotriaosylceramide	142-163	galactosidase alpha	Homo sapiens	93-104
26291612-1	2015	Fabry disease is a lysosomal storage disorder caused by deficiency of alpha-galactosidase A (alpha-gal A), which results in the deposition of globotriaosylceramide (Gb3) in the vascular endothelium.	globotriaosylceramide	142-163	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	165-168
26004871-0	2015	Targeting glucosylceramide synthase induction of cell surface globotriaosylceramide (Gb3) in acquired cisplatin-resistance of lung cancer and malignant pleural mesothelioma cells.	globotriaosylceramide	62-83	UDP-glucose ceramide glucosyltransferase	Homo sapiens	10-35
26004871-0	2015	Targeting glucosylceramide synthase induction of cell surface globotriaosylceramide (Gb3) in acquired cisplatin-resistance of lung cancer and malignant pleural mesothelioma cells.	globotriaosylceramide	62-83	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	85-88
26004871-3	2015	However, a tumour stress activation of glucosylceramide synthase (GCS) follows to eliminate ceramide by formation of glycosphingolipids (GSLs) such as globotriaosylceramide (Gb3), the functional receptor of verotoxin-1.	globotriaosylceramide	151-172	UDP-glucose ceramide glucosyltransferase	Homo sapiens	39-64
26004871-3	2015	However, a tumour stress activation of glucosylceramide synthase (GCS) follows to eliminate ceramide by formation of glycosphingolipids (GSLs) such as globotriaosylceramide (Gb3), the functional receptor of verotoxin-1.	globotriaosylceramide	151-172	UDP-glucose ceramide glucosyltransferase	Homo sapiens	66-69
25915924-1	2015	Fabry disease is an X-linked lysosomal storage disorder caused by mutations in the gene that encodes alpha-galactosidase A and is characterized by pathological accumulation of globotriaosylceramide and globotriaosylsphingosine.	globotriaosylceramide	176-197	galactosidase alpha	Homo sapiens	101-122
25697597-2	2015	Globotriaosylsphingosine (lyso-Gb3), a deacylated form of globotriaosylceramide (Gb3), has emerged as a marker of Fabry disease.	globotriaosylceramide	58-79	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	81-84
25030479-1	2015	Anderson-Fabry disease (AFD) is a lysosomal storage disease caused by the inappropriate accumulation of globotriaosylceramide in tissues due to a deficiency in the enzyme alpha-galactosidase A (alpha-Gal A).	globotriaosylceramide	104-125	galactosidase alpha	Homo sapiens	171-192
25030479-1	2015	Anderson-Fabry disease (AFD) is a lysosomal storage disease caused by the inappropriate accumulation of globotriaosylceramide in tissues due to a deficiency in the enzyme alpha-galactosidase A (alpha-Gal A).	globotriaosylceramide	104-125	galactosidase alpha	Homo sapiens	194-205
25156739-6	2014	Since the glycosphingolipids (GSLs) globotriaosylceramide (Gb3Cer) and globotetraosylceramide (Gb4Cer) are well-known receptors for Stx but also for P fimbriae, a major virulence factor of extraintestinal pathogenic E. coli (ExPEC), the expression of Gb3Cer and Gb4Cer by T24 cells and in murine urinary bladder tissue was examined by thin-layer chromatography and mass spectrometry.	globotriaosylceramide	36-57	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	59-62
25690728-2	2015	This defect results in an accumulation of glycosphingolipids, primarily globotriaosylceramide (Gb3) which causes a multisystemic vasculopathy.	globotriaosylceramide	72-93	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	95-98
25701267-2	2015	Reduced or missing alpha-gal A enzyme results in the storage of globotriaosylceramide (GL3) and related glycosphingolipids in the cellular lysosomes throughout the body.	globotriaosylceramide	64-85	galactosidase alpha	Homo sapiens	19-30
25386848-2	2014	an X-linked deficiency of alpha-galactosidase A coded by the GLA gene, leads to intracellular globotriaosylceramide (GL-3) accumulation.	globotriaosylceramide	94-115	galactosidase alpha	Homo sapiens	26-47
25386848-2	2014	an X-linked deficiency of alpha-galactosidase A coded by the GLA gene, leads to intracellular globotriaosylceramide (GL-3) accumulation.	globotriaosylceramide	94-115	galactosidase alpha	Homo sapiens	61-64
25637016-5	2015	Stx2 bound to P1 (k) and P2 (k) phenotype RBCs, expressing high levels of the P(k) Ag (globotriaosylceramide), the known Stx receptor.	globotriaosylceramide	87-108	syntaxin 2	Homo sapiens	0-4
25575293-1	2015	Globotriaosylceramide (Gb3) is a glycosphingolipid present in the plasma membrane that is the natural receptor of the bacterial Shiga toxin.	globotriaosylceramide	0-21	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	23-26
25156739-6	2014	Since the glycosphingolipids (GSLs) globotriaosylceramide (Gb3Cer) and globotetraosylceramide (Gb4Cer) are well-known receptors for Stx but also for P fimbriae, a major virulence factor of extraintestinal pathogenic E. coli (ExPEC), the expression of Gb3Cer and Gb4Cer by T24 cells and in murine urinary bladder tissue was examined by thin-layer chromatography and mass spectrometry.	globotriaosylceramide	36-57	beta-1,3-N-acetylgalactosaminyltransferase 1 (globoside blood group)	Homo sapiens	262-268
25185550-1	2014	Globotriaosylceramide (Gb3), a glycosphingolipid found in the plasma membrane of animal cells, is the endocytic receptor of the bacterial Shiga toxin.	globotriaosylceramide	0-21	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	23-26
25337704-2	2014	The alpha-GalA deficiency leads to multi-systemic clinical manifestations caused by the preferential accumulation of globotriaosylceramide in the endothelium and vascular smooth muscles.	globotriaosylceramide	117-138	galactosidase, alpha	Mus musculus	4-14
24645664-1	2014	Fabry disease (FD) is an X-linked disease in which mutations of the GLA gene result in a deficiency of the enzyme alpha-galactosidase A and subsequent progressive, intralysosomal deposition of undegraded glycosphingolipid products, primarily globotriaosylceramide, in multiple organs.	globotriaosylceramide	242-263	galactosidase alpha	Homo sapiens	68-71
24778057-1	2014	Fabry disease is an inherited (X-linked) lysosomal storage disorder caused by deficiency of alpha-galactosidase A, leading to accumulation of globotriaosylceramide in various tissues.	globotriaosylceramide	142-163	galactosidase alpha	Homo sapiens	92-113
25345090-1	2014	Fabry disease (FD) is an X-linked disorder caused by deficiency of the enzyme alpha-galactosidase A, required for the degradation of globotriaosylceramide.	globotriaosylceramide	133-154	galactosidase alpha	Homo sapiens	78-99
24886109-2	2014	Complete or partial deficiency in this enzyme leads to intracellular accumulation of globotriaosylceramide (Gb3) and related glycosphingolipids in many cell types throughout the body, including the kidney.	globotriaosylceramide	85-106	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	108-111
25530917-7	2014	The function of the B pentamer is to bind to the cellular receptor, globotriaosylceramide, Gb3, found primarily on endothelial cells.	globotriaosylceramide	68-89	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	91-94
24402087-5	2014	Knockdown of alpha-galactosidase A was confirmed using immunoblotting and globotriaosylceramide accumulation.	globotriaosylceramide	74-95	galactosidase alpha	Homo sapiens	13-34
24980630-4	2014	Except for three patients who had received ERT for more than 3 years, all other patients showed significant pathological change and globotriaosylceramide (Gb3) accumulation in their cardiomyocytes.	globotriaosylceramide	132-153	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	155-158
24983355-5	2014	Here, we studied binding of holotoxin and B-subunits of Stx1, Stx2a, Stx2b, Stx2c and Stx2d to glycolipid receptors globotriaosylceramide (Gb3) and globotetraosylceramide (Gb4) in the presence of cell membrane components such as phosphatidylcholine (PC), cholesterol (Ch) and other neutral glycolipids.	globotriaosylceramide	116-137	syntaxin 1A	Homo sapiens	56-60
24983355-5	2014	Here, we studied binding of holotoxin and B-subunits of Stx1, Stx2a, Stx2b, Stx2c and Stx2d to glycolipid receptors globotriaosylceramide (Gb3) and globotetraosylceramide (Gb4) in the presence of cell membrane components such as phosphatidylcholine (PC), cholesterol (Ch) and other neutral glycolipids.	globotriaosylceramide	116-137	syntaxin 2	Homo sapiens	62-67
24983355-5	2014	Here, we studied binding of holotoxin and B-subunits of Stx1, Stx2a, Stx2b, Stx2c and Stx2d to glycolipid receptors globotriaosylceramide (Gb3) and globotetraosylceramide (Gb4) in the presence of cell membrane components such as phosphatidylcholine (PC), cholesterol (Ch) and other neutral glycolipids.	globotriaosylceramide	116-137	syntaxin 2	Homo sapiens	69-74
24983355-5	2014	Here, we studied binding of holotoxin and B-subunits of Stx1, Stx2a, Stx2b, Stx2c and Stx2d to glycolipid receptors globotriaosylceramide (Gb3) and globotetraosylceramide (Gb4) in the presence of cell membrane components such as phosphatidylcholine (PC), cholesterol (Ch) and other neutral glycolipids.	globotriaosylceramide	116-137	syntaxin 2	Homo sapiens	76-81
26012164-1	2014	Fabry disease (Anderson-Fabry disease) is one of the most common lysosomal storage diseases (after Gaucher disease) caused by deficient activity of the alpha-galactosidase A (alpha-Gal A) enzyme, which leads to progressive accumulation of globotriaosylceramide in various cells, predominantly in endothelium and vascular smooth muscles, with multisystem clinical manifestations.	globotriaosylceramide	239-260	galactosidase alpha	Homo sapiens	152-173
26012164-1	2014	Fabry disease (Anderson-Fabry disease) is one of the most common lysosomal storage diseases (after Gaucher disease) caused by deficient activity of the alpha-galactosidase A (alpha-Gal A) enzyme, which leads to progressive accumulation of globotriaosylceramide in various cells, predominantly in endothelium and vascular smooth muscles, with multisystem clinical manifestations.	globotriaosylceramide	239-260	galactosidase alpha	Homo sapiens	175-186
25530762-1	2014	Fabry disease is an X-linked lysosomal storage disorder caused by deficient activity of a-galactosidase A (also known as ceramide trihexosidase) and resultant accumulation of globotriaosylceramide (Gb3) and related glycophospholipids.	globotriaosylceramide	175-196	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	198-201
24634980-1	2014	Fabry disease is a multisystemic, X-linked lysosomal storage disorder caused by a deficit in alpha-galactosidase A enzyme activity leading to glycosphingolipid accumulation, mainly globotriaosylceramide (Gb3) and globotriaosylsphingosine (lyso-Gb3).	globotriaosylceramide	181-202	galactosidase alpha	Homo sapiens	93-114
24634980-1	2014	Fabry disease is a multisystemic, X-linked lysosomal storage disorder caused by a deficit in alpha-galactosidase A enzyme activity leading to glycosphingolipid accumulation, mainly globotriaosylceramide (Gb3) and globotriaosylsphingosine (lyso-Gb3).	globotriaosylceramide	181-202	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	204-207
24671194-3	2014	Although Stx1a and Stx2a bind to the same receptor, globotriaosylceramide (Gb3), Stx2a is more potent than Stx1a in mice, whereas Stx1a is more cytotoxic than Stx2a in cell culture.	globotriaosylceramide	52-73	syntaxin 1A (brain)	Mus musculus	9-14
24215016-2	2014	Deficiency of alpha-galactosidase A (alpha-Gal A) causes intracellular accumulations of globotriaosylceramide (GL-3) and related glycosphingolipids in all organs, including the kidney, often leading to end-stage renal failure.	globotriaosylceramide	88-109	galactosidase alpha	Homo sapiens	14-35
24215016-2	2014	Deficiency of alpha-galactosidase A (alpha-Gal A) causes intracellular accumulations of globotriaosylceramide (GL-3) and related glycosphingolipids in all organs, including the kidney, often leading to end-stage renal failure.	globotriaosylceramide	88-109	galactosidase alpha	Homo sapiens	37-48
24626231-8	2014	A myocardial biopsy performed in one during this procedure demonstrated substantial accumulation of globotriaosylceramide (Gb3) in cardiomyocytes.	globotriaosylceramide	100-121	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	123-126
24215843-1	2014	Accumulation of globotriaosylceramide (Gb3) and other neutral glycosphingolipids with galactosyl residues is the hallmark of Fabry disease, a lysosomal storage disorder caused by deficiency of the enzyme alpha-galactosidase A (alpha-gal A).	globotriaosylceramide	16-37	galactosidase alpha	Canis lupus familiaris	204-225
24215843-1	2014	Accumulation of globotriaosylceramide (Gb3) and other neutral glycosphingolipids with galactosyl residues is the hallmark of Fabry disease, a lysosomal storage disorder caused by deficiency of the enzyme alpha-galactosidase A (alpha-gal A).	globotriaosylceramide	16-37	galactosidase alpha	Canis lupus familiaris	227-238
24215843-1	2014	Accumulation of globotriaosylceramide (Gb3) and other neutral glycosphingolipids with galactosyl residues is the hallmark of Fabry disease, a lysosomal storage disorder caused by deficiency of the enzyme alpha-galactosidase A (alpha-gal A).	globotriaosylceramide	39-42	galactosidase alpha	Canis lupus familiaris	204-225
24215843-1	2014	Accumulation of globotriaosylceramide (Gb3) and other neutral glycosphingolipids with galactosyl residues is the hallmark of Fabry disease, a lysosomal storage disorder caused by deficiency of the enzyme alpha-galactosidase A (alpha-gal A).	globotriaosylceramide	39-42	galactosidase alpha	Canis lupus familiaris	227-238
24398019-1	2014	BACKGROUND: Fabry disease is an X-linked inherited metabolic condition where the deficit of the alpha-galactosidase A enzyme, encoded by the GLA gene, leads to glycosphingolipid storage, mainly globotriaosylceramide.	globotriaosylceramide	194-215	galactosidase alpha	Homo sapiens	96-117
24398019-1	2014	BACKGROUND: Fabry disease is an X-linked inherited metabolic condition where the deficit of the alpha-galactosidase A enzyme, encoded by the GLA gene, leads to glycosphingolipid storage, mainly globotriaosylceramide.	globotriaosylceramide	194-215	galactosidase alpha	Homo sapiens	141-144
24232002-1	2014	A defect in the gene for the lysosomal enzyme alpha-galactosidase A (Gla) results in globotriaosylceramide (Gb3) accumulation in Fabry disease and leads to premature death from cardiac and cerebrovascular events.	globotriaosylceramide	85-106	galactosidase, alpha	Mus musculus	46-67
24232002-1	2014	A defect in the gene for the lysosomal enzyme alpha-galactosidase A (Gla) results in globotriaosylceramide (Gb3) accumulation in Fabry disease and leads to premature death from cardiac and cerebrovascular events.	globotriaosylceramide	85-106	galactosidase, alpha	Mus musculus	69-72
24232002-1	2014	A defect in the gene for the lysosomal enzyme alpha-galactosidase A (Gla) results in globotriaosylceramide (Gb3) accumulation in Fabry disease and leads to premature death from cardiac and cerebrovascular events.	globotriaosylceramide	108-111	galactosidase, alpha	Mus musculus	46-67
24232002-1	2014	A defect in the gene for the lysosomal enzyme alpha-galactosidase A (Gla) results in globotriaosylceramide (Gb3) accumulation in Fabry disease and leads to premature death from cardiac and cerebrovascular events.	globotriaosylceramide	108-111	galactosidase, alpha	Mus musculus	69-72
24158513-0	2014	Globotriaosylceramide induces lysosomal degradation of endothelial KCa3.1 in fabry disease.	globotriaosylceramide	0-21	potassium intermediate/small conductance calcium-activated channel, subfamily N, member 4	Mus musculus	67-73
24158513-1	2014	OBJECTIVE: Globotriaosylceramide (Gb3) induces KCa3.1 downregulation in Fabry disease (FD).	globotriaosylceramide	11-32	potassium intermediate/small conductance calcium-activated channel, subfamily N, member 4	Mus musculus	47-53
24158513-1	2014	OBJECTIVE: Globotriaosylceramide (Gb3) induces KCa3.1 downregulation in Fabry disease (FD).	globotriaosylceramide	34-37	potassium intermediate/small conductance calcium-activated channel, subfamily N, member 4	Mus musculus	47-53
23690406-1	2013	The two major forms of Shiga toxin, Stx1 and Stx2, use the glycolipid globotriaosylceramide (Gb3) as their cellular receptor.	globotriaosylceramide	70-91	syntaxin 1A	Homo sapiens	36-40
23906628-5	2013	In ovarian carcinoma, globotriaosyl ceramide (Gb3), the GSL receptor for the antineoplastic Escherichia coli-derived verotoxin, was increased throughout the tumor.	globotriaosylceramide	22-44	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	46-49
23906628-5	2013	In ovarian carcinoma, globotriaosyl ceramide (Gb3), the GSL receptor for the antineoplastic Escherichia coli-derived verotoxin, was increased throughout the tumor.	globotriaosylceramide	22-44	cathepsin A	Homo sapiens	56-59
24526817-0	2013	Spontaneous Accumulation of Globotriaosylceramide (Gb3) in Proximal Renal Tubules in an ICR Mouse.	globotriaosylceramide	28-49	CD1 antigen complex	Mus musculus	88-91
24526817-0	2013	Spontaneous Accumulation of Globotriaosylceramide (Gb3) in Proximal Renal Tubules in an ICR Mouse.	globotriaosylceramide	51-54	CD1 antigen complex	Mus musculus	88-91
23968398-2	2013	In addition to the two biomarkers, globotriaosylceramide (Gb3) and globotriaosylsphingosine (lyso-Gb3), which are routinely used for detection and high-risk screening of Fabry disease patients, novel urinary Gb3-related isoforms/analogues as well as newly defined lyso-Gb3 analogues in plasma and urine from Fabry patients have recently been described by our group.	globotriaosylceramide	35-56	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	58-61
24558776-1	2013	Fabry disease (Anderson-Fabry disease) is one of the most common lysosomal storage diseases (after Gaucher disease) caused by deficient activity of the alpha-galactosidase A (alpha-Gal A) enzyme, which leads to progressive accumulation of globotriaosylceramide in various cells, predominantly in endothelium and vascular smooth muscles, with multisystem clinical manifestations.	globotriaosylceramide	239-260	galactosidase alpha	Homo sapiens	152-173
24558776-1	2013	Fabry disease (Anderson-Fabry disease) is one of the most common lysosomal storage diseases (after Gaucher disease) caused by deficient activity of the alpha-galactosidase A (alpha-Gal A) enzyme, which leads to progressive accumulation of globotriaosylceramide in various cells, predominantly in endothelium and vascular smooth muscles, with multisystem clinical manifestations.	globotriaosylceramide	239-260	galactosidase alpha	Homo sapiens	175-186
23690406-1	2013	The two major forms of Shiga toxin, Stx1 and Stx2, use the glycolipid globotriaosylceramide (Gb3) as their cellular receptor.	globotriaosylceramide	70-91	syntaxin 2	Homo sapiens	45-49
23690406-1	2013	The two major forms of Shiga toxin, Stx1 and Stx2, use the glycolipid globotriaosylceramide (Gb3) as their cellular receptor.	globotriaosylceramide	70-91	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	93-96
23702393-6	2013	A trend toward increased sweat volume on QSART testing, and reduced urine globotriaosylceramide concentration were seen with treatment schedule C. Agalsidase alfa was safe and well tolerated with all schedules.	globotriaosylceramide	74-95	galactosidase alpha	Homo sapiens	147-162
23474038-1	2013	BACKGROUND: Fabry disease (FD) is a genetic disorder resulting from deficiency of the lysosomal enzyme alpha-galactosidase A (alpha-Gal A) which leads to globotriaosylceramide (GL-3) accumulation in multiple tissues.	globotriaosylceramide	154-175	galactosidase alpha	Homo sapiens	103-124
23385635-2	2013	The deficiency in alpha-GalA activity leads to an intra-lysosomal accumulation of neutral glycosphingolipids, mainly globotriaosylceramide (Gb3), in various organs and systems.	globotriaosylceramide	117-138	galactosidase alpha	Homo sapiens	18-28
23385635-2	2013	The deficiency in alpha-GalA activity leads to an intra-lysosomal accumulation of neutral glycosphingolipids, mainly globotriaosylceramide (Gb3), in various organs and systems.	globotriaosylceramide	117-138	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	140-143
23691056-2	2013	This X-linked defect results in the accumulation of enzyme substrates with terminally alpha-glycosidically bound galactose, mainly the neutral glycosphingolipid Globotriaosylceramide (Gb3) in various tissues, including the kidneys.	globotriaosylceramide	161-182	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	184-187
23452955-1	2013	Fabry disease is an X-linked lysosomal disorder (LD) due to deficiency of the enzyme alpha-galactosidase A (alphaGal), which leads to the accumulation of neutral glycosphingolipids, mainly globotriaosylceramide (Gb3).	globotriaosylceramide	189-210	galactosidase alpha	Homo sapiens	85-106
23474038-1	2013	BACKGROUND: Fabry disease (FD) is a genetic disorder resulting from deficiency of the lysosomal enzyme alpha-galactosidase A (alpha-Gal A) which leads to globotriaosylceramide (GL-3) accumulation in multiple tissues.	globotriaosylceramide	154-175	galactosidase alpha	Homo sapiens	126-137
22766973-2	2013	Stxs are AB(5) toxins composed of an enzymatically active A subunit and the pentameric B subunit, which preferentially binds to the glycosphingolipid globotriaosylceramide (Gb3Cer/CD77).	globotriaosylceramide	150-171	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	180-184
23433711-2	2013	In Fabry disease there is accumulation of mainly globotriaosylceramide due to deficiency of the lysosomal enzyme alpha-galactosidase A.	globotriaosylceramide	49-70	galactosidase alpha	Homo sapiens	113-134
23734323-2	2013	We proposed globotriaosylceramide (Gb3) as a viable alternative target for antiangiogenic therapies.	globotriaosylceramide	12-33	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	35-38
23380967-2	2013	Therefore, Gb(3) in resting or activated CD4(+) T-cells was assessed by flow cytometry and thin layer chromatography of cell extracts.	globotriaosylceramide	11-16	CD4 molecule	Homo sapiens	41-44
23334311-1	2013	Fabry disease (FD) is an X-linked lysosomal storage disorder caused by accumulation of Gb-3 (globotriaosylceramide) in cellular lysosomes of tissues throughout the body.	globotriaosylceramide	93-114	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	87-91
23448451-2	2013	FD causes glycolipids, such as globotriaosylceramide (Gb3), to accumulate in the vascular endothelium of several organs (Fig.	globotriaosylceramide	31-52	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	54-57
23176611-1	2012	BACKGROUND: Fabry disease (FD) is a genetic disorder resulting from deficiency of the lysosomal enzyme alpha-galactosidase A (alpha-Gal A), which leads to globotriaosylceramide (GL-3) accumulation in multiple tissues.	globotriaosylceramide	155-176	galactosidase alpha	Homo sapiens	103-124
23472096-1	2013	Fabry disease (FD) results from mutations in the gene (GLA) that encodes the lysosomal enzyme alpha-galactosidase A (alpha-Gal A), and involves pathological accumulation of globotriaosylceramide (GL-3) and globotriaosylsphingosine (lyso-Gb3).	globotriaosylceramide	173-194	galactosidase alpha	Homo sapiens	55-58
23472096-1	2013	Fabry disease (FD) results from mutations in the gene (GLA) that encodes the lysosomal enzyme alpha-galactosidase A (alpha-Gal A), and involves pathological accumulation of globotriaosylceramide (GL-3) and globotriaosylsphingosine (lyso-Gb3).	globotriaosylceramide	173-194	galactosidase alpha	Homo sapiens	94-115
23472096-1	2013	Fabry disease (FD) results from mutations in the gene (GLA) that encodes the lysosomal enzyme alpha-galactosidase A (alpha-Gal A), and involves pathological accumulation of globotriaosylceramide (GL-3) and globotriaosylsphingosine (lyso-Gb3).	globotriaosylceramide	173-194	galactosidase alpha	Homo sapiens	117-128
23093409-6	2012	Frontal affinity chromatography technology indicated that MytiLec bound specifically to globotriose (Gb3; Galalpha1-4Galbeta1-4Glc), the epitope of globotriaosylceramide.	globotriaosylceramide	148-169	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	101-104
23242319-2	2012	Gb(3) is a B cell marker (CD77), but a fraction of activated peripheral blood mononuclear cells (PBMCs) can also express Gb(3).	globotriaosylceramide	0-5	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	26-30
23007467-1	2012	Fabry disease is a lysosomal storage disorder (LSD) caused by deficiency             of alpha-galactosidase A (alpha-gal A), resulting in deposition of globotriaosylceramide             (Gb3; also known as ceramide trihexoside) in the vascular endothelium of many             organs.	globotriaosylceramide	152-173	galactosidase, alpha	Mus musculus	88-109
23007467-1	2012	Fabry disease is a lysosomal storage disorder (LSD) caused by deficiency             of alpha-galactosidase A (alpha-gal A), resulting in deposition of globotriaosylceramide             (Gb3; also known as ceramide trihexoside) in the vascular endothelium of many             organs.	globotriaosylceramide	152-173	galactosidase, alpha	Mus musculus	111-122
23007467-1	2012	Fabry disease is a lysosomal storage disorder (LSD) caused by deficiency             of alpha-galactosidase A (alpha-gal A), resulting in deposition of globotriaosylceramide             (Gb3; also known as ceramide trihexoside) in the vascular endothelium of many             organs.	globotriaosylceramide	187-190	galactosidase, alpha	Mus musculus	88-109
23007467-1	2012	Fabry disease is a lysosomal storage disorder (LSD) caused by deficiency             of alpha-galactosidase A (alpha-gal A), resulting in deposition of globotriaosylceramide             (Gb3; also known as ceramide trihexoside) in the vascular endothelium of many             organs.	globotriaosylceramide	187-190	galactosidase, alpha	Mus musculus	111-122
23176611-1	2012	BACKGROUND: Fabry disease (FD) is a genetic disorder resulting from deficiency of the lysosomal enzyme alpha-galactosidase A (alpha-Gal A), which leads to globotriaosylceramide (GL-3) accumulation in multiple tissues.	globotriaosylceramide	155-176	galactosidase alpha	Homo sapiens	126-137
23330407-1	2012	Fabry disease (Anderson-Fabry disease) is an X-linked recessive lysosomal storage disorder resulting from deficient activity of lysosomal hydrolase, alpha-galactosidase A (alpha-Gal A), which leads to progressive accumulation of globotriaosylceramide (Gb3) in various cells, predominantly endothelial and vascular smooth muscle cells, with clinical manifestations affecting major organs including the central nervous system.	globotriaosylceramide	229-250	galactosidase alpha	Homo sapiens	149-170
23058197-1	2012	UNLABELLED: Fabry disease (FD) is a rare X-linked genetic lysosomal storage disease caused by a deficiency of the enzyme alpha-galactosidase A, that produces accumulation of globotriaosylceramide.	globotriaosylceramide	174-195	galactosidase alpha	Homo sapiens	121-142
26019818-2	2012	Scarce activity of lysosomal alpha-galactosidase A results in progressive accumulation of globotriaosylceramide (Gb3) within lysosomes, believed to trigger a flow of cellular changes that lead to the clinical manifestation of the disease.	globotriaosylceramide	90-111	galactosidase alpha	Homo sapiens	29-50
26019818-2	2012	Scarce activity of lysosomal alpha-galactosidase A results in progressive accumulation of globotriaosylceramide (Gb3) within lysosomes, believed to trigger a flow of cellular changes that lead to the clinical manifestation of the disease.	globotriaosylceramide	90-111	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	113-116
22936806-4	2012	Enhanced ceramide glycosylation and globotriosylceramide (Gb3) correlate well with the numbers of BCSCs in breast cancer cell lines.	globotriaosylceramide	36-56	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	58-61
23330407-1	2012	Fabry disease (Anderson-Fabry disease) is an X-linked recessive lysosomal storage disorder resulting from deficient activity of lysosomal hydrolase, alpha-galactosidase A (alpha-Gal A), which leads to progressive accumulation of globotriaosylceramide (Gb3) in various cells, predominantly endothelial and vascular smooth muscle cells, with clinical manifestations affecting major organs including the central nervous system.	globotriaosylceramide	229-250	galactosidase alpha	Homo sapiens	172-183
22187137-1	2012	Fabry disease (FD) is an X-linked inherited disease due to alpha-galactosidase A (alpha-Gal A) deficiency and characterized by lysosomal storage of globotriaosylceramide (Gb3) and related neutral glycosphingolipids.	globotriaosylceramide	148-169	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	171-174
22472949-1	2012	Fabry disease is a lysosomal storage disorder caused by a deficiency of alpha-galactosidase A (alpha-gal A) activity that results in progressive globotriaosylceramide (Gb(3)) deposition.	globotriaosylceramide	145-166	galactosidase alpha	Homo sapiens	72-93
22472949-1	2012	Fabry disease is a lysosomal storage disorder caused by a deficiency of alpha-galactosidase A (alpha-gal A) activity that results in progressive globotriaosylceramide (Gb(3)) deposition.	globotriaosylceramide	145-166	galactosidase alpha	Homo sapiens	95-106
22472949-1	2012	Fabry disease is a lysosomal storage disorder caused by a deficiency of alpha-galactosidase A (alpha-gal A) activity that results in progressive globotriaosylceramide (Gb(3)) deposition.	globotriaosylceramide	168-174	galactosidase alpha	Homo sapiens	72-93
22472949-1	2012	Fabry disease is a lysosomal storage disorder caused by a deficiency of alpha-galactosidase A (alpha-gal A) activity that results in progressive globotriaosylceramide (Gb(3)) deposition.	globotriaosylceramide	168-174	galactosidase alpha	Homo sapiens	95-106
22425450-1	2012	Fabry disease is a lysosomal storage disease caused by deficient activity of the alpha-Galactosidase A (alpha-Gal A) enzyme, which leads to abnormal accumulation of glycosphingolipids, mainly globotriaosylceramide (Gb3), in the lysosome.	globotriaosylceramide	192-213	galactosidase, alpha	Mus musculus	81-102
22425450-1	2012	Fabry disease is a lysosomal storage disease caused by deficient activity of the alpha-Galactosidase A (alpha-Gal A) enzyme, which leads to abnormal accumulation of glycosphingolipids, mainly globotriaosylceramide (Gb3), in the lysosome.	globotriaosylceramide	192-213	galactosidase, alpha	Mus musculus	104-115
22425450-1	2012	Fabry disease is a lysosomal storage disease caused by deficient activity of the alpha-Galactosidase A (alpha-Gal A) enzyme, which leads to abnormal accumulation of glycosphingolipids, mainly globotriaosylceramide (Gb3), in the lysosome.	globotriaosylceramide	192-213	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	215-218
22309310-2	2012	Two disease-specific Fabry biomarkers have been identified and quantified in plasma and urine: globotriaosylceramide (Gb(3)) and globotriaosylsphingosine (lyso-Gb(3)).	globotriaosylceramide	95-116	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	118-123
22215019-1	2012	Fabry disease is an X-linked lysosomal storage disorder (LSD) caused by mutations in the gene (GLA) that encodes the lysosomal hydrolase alpha-galactosidase A (alpha-Gal A), and is characterized by pathological accumulation of the substrate, globotriaosylceramide (GL-3).	globotriaosylceramide	242-263	galactosidase alpha	Homo sapiens	95-98
22215019-1	2012	Fabry disease is an X-linked lysosomal storage disorder (LSD) caused by mutations in the gene (GLA) that encodes the lysosomal hydrolase alpha-galactosidase A (alpha-Gal A), and is characterized by pathological accumulation of the substrate, globotriaosylceramide (GL-3).	globotriaosylceramide	242-263	galactosidase alpha	Homo sapiens	137-158
22215019-1	2012	Fabry disease is an X-linked lysosomal storage disorder (LSD) caused by mutations in the gene (GLA) that encodes the lysosomal hydrolase alpha-galactosidase A (alpha-Gal A), and is characterized by pathological accumulation of the substrate, globotriaosylceramide (GL-3).	globotriaosylceramide	242-263	galactosidase alpha	Homo sapiens	160-171
22085605-1	2012	Fabry disease is an X-linked inborn error of glycosphingolipid catabolism due to deficient activity of alpha-galactosidase A that leads to accumulation of the enzyme substrates, mainly globotriaosylceramide (Gb3), in body fluids and lysosomes of many cell types.	globotriaosylceramide	185-206	galactosidase alpha	Homo sapiens	103-124
23766930-1	2012	We have previously demonstrated an association between the accumulation of the glycosphingolipid globotriaosylceramide (Gb3) and the loss of high molecular weight oligomers in the aortas of alpha-galactosidase A-knockout mice, a model of Fabry disease.	globotriaosylceramide	97-118	galactosidase, alpha	Mus musculus	190-211
22085605-1	2012	Fabry disease is an X-linked inborn error of glycosphingolipid catabolism due to deficient activity of alpha-galactosidase A that leads to accumulation of the enzyme substrates, mainly globotriaosylceramide (Gb3), in body fluids and lysosomes of many cell types.	globotriaosylceramide	185-206	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	208-211
22205110-1	2012	Fabry disease (FD) is a rare X-linked lysosomal storage disorder of glycosphingolipids, mostly globotriaosylceramide (Gb3).	globotriaosylceramide	95-116	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	118-121
22138589-2	2012	A defect in the alpha-galactosidase gene leads to lysosomal accumulation of the glycolipid globotriaosylceramide (Gb3).	globotriaosylceramide	91-112	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	114-117
23208084-6	2012	The link connected the accumulation of glycosphingolipid globotriaosylceramide, characteristic of FD, with the expression of CD74 and macrophage migration inhibitory factor that may play an important role in tumorigenesis regulated by the Von Hippel-Lindau/hypoxia-inducible factor 1alpha pathway.	globotriaosylceramide	57-78	CD74 molecule	Homo sapiens	125-129
22612023-1	2012	Fabry disease is an X-linked inherited condition with the absence or reduction of alpha-galactosidase A- activity in lysosomes leading to accumulation of globotriaosylceramide and related neutral glycosphingolipids.	globotriaosylceramide	154-175	galactosidase alpha	Homo sapiens	82-103
23304632-7	2012	Immunohistochemistry and real-time PCR revealed that the expression of globotriaosylceramide (Gb3), the receptor for Stx2, and Gb3 synthase (GalT6) in HASTs was increased by interleukin-1beta (IL-1beta).	globotriaosylceramide	71-92	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	94-97
23094092-2	2012	Anti agalsidase ABs negatively influence globotriaosylceramide (Gb3) reduction.	globotriaosylceramide	41-62	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	64-67
23304632-7	2012	Immunohistochemistry and real-time PCR revealed that the expression of globotriaosylceramide (Gb3), the receptor for Stx2, and Gb3 synthase (GalT6) in HASTs was increased by interleukin-1beta (IL-1beta).	globotriaosylceramide	71-92	syntaxin 2	Homo sapiens	117-121
23304632-7	2012	Immunohistochemistry and real-time PCR revealed that the expression of globotriaosylceramide (Gb3), the receptor for Stx2, and Gb3 synthase (GalT6) in HASTs was increased by interleukin-1beta (IL-1beta).	globotriaosylceramide	71-92	interleukin 1 beta	Homo sapiens	174-191
23304632-7	2012	Immunohistochemistry and real-time PCR revealed that the expression of globotriaosylceramide (Gb3), the receptor for Stx2, and Gb3 synthase (GalT6) in HASTs was increased by interleukin-1beta (IL-1beta).	globotriaosylceramide	71-92	interleukin 1 beta	Homo sapiens	193-201
22563919-1	2012	Fabry disease--a genetic disorder characterized by the accumulation of globotriaosylceramide in cell lysosomes resulting from an X-linked deficiency of alpha-galactosidase A activity--presents with multiorgan manifestations, including progressive renal disease.	globotriaosylceramide	71-92	galactosidase alpha	Homo sapiens	152-173
22241068-4	2012	The oral administration of 1-deoxygalactonojirimycin to transgenic mice expressing human mutant alpha-galactosidase A resulted in significant increases in alpha-galactosidase A activity in various organs, with concomitant reductions in globotriaosylceramide, which contributes to the pathology of Fabry disease.	globotriaosylceramide	236-257	galactosidase alpha	Homo sapiens	96-117
21896204-1	2011	BACKGROUND: Anderson-Fabry disease (FD) is caused by a deficit of the alpha-galactosidase A enzyme which leads to the accumulation of complex sphingolipids, especially globotriaosylceramide (Gb3), in all the cells of the body, causing the onset of a multi-systemic disease with poor prognosis in adulthood.	globotriaosylceramide	168-189	galactosidase alpha	Homo sapiens	70-91
22472932-2	2011	Fabry disease is an X-linked hereditary metabolic storage disorder, due to the deficiency in lysosomal alpha-galactosidase A, with the consequent glycosphingolipids accumulation, primarily globotriaosylceramide, at cellular level.	globotriaosylceramide	189-210	galactosidase alpha	Homo sapiens	103-124
21069478-3	2011	Natural glycosphingolipid, globotriaosylceramide (Gal alpha1-4, Gal beta1-4, Glc beta1-1, ceramide), is also called CD77 and its expression was previously associated with proliferating centroblasts undergoing somatic hypermutation, but it has been demonstrate that globotriaosylceramide is not a reliable marker to discriminate human centroblasts from centrocytes.	globotriaosylceramide	27-48	UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 2	Homo sapiens	68-75
21069478-3	2011	Natural glycosphingolipid, globotriaosylceramide (Gal alpha1-4, Gal beta1-4, Glc beta1-1, ceramide), is also called CD77 and its expression was previously associated with proliferating centroblasts undergoing somatic hypermutation, but it has been demonstrate that globotriaosylceramide is not a reliable marker to discriminate human centroblasts from centrocytes.	globotriaosylceramide	27-48	UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 2	Homo sapiens	81-88
21069478-3	2011	Natural glycosphingolipid, globotriaosylceramide (Gal alpha1-4, Gal beta1-4, Glc beta1-1, ceramide), is also called CD77 and its expression was previously associated with proliferating centroblasts undergoing somatic hypermutation, but it has been demonstrate that globotriaosylceramide is not a reliable marker to discriminate human centroblasts from centrocytes.	globotriaosylceramide	27-48	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	116-120
21069478-3	2011	Natural glycosphingolipid, globotriaosylceramide (Gal alpha1-4, Gal beta1-4, Glc beta1-1, ceramide), is also called CD77 and its expression was previously associated with proliferating centroblasts undergoing somatic hypermutation, but it has been demonstrate that globotriaosylceramide is not a reliable marker to discriminate human centroblasts from centrocytes.	globotriaosylceramide	265-286	UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 2	Homo sapiens	68-75
21069478-3	2011	Natural glycosphingolipid, globotriaosylceramide (Gal alpha1-4, Gal beta1-4, Glc beta1-1, ceramide), is also called CD77 and its expression was previously associated with proliferating centroblasts undergoing somatic hypermutation, but it has been demonstrate that globotriaosylceramide is not a reliable marker to discriminate human centroblasts from centrocytes.	globotriaosylceramide	265-286	UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 2	Homo sapiens	81-88
21069478-3	2011	Natural glycosphingolipid, globotriaosylceramide (Gal alpha1-4, Gal beta1-4, Glc beta1-1, ceramide), is also called CD77 and its expression was previously associated with proliferating centroblasts undergoing somatic hypermutation, but it has been demonstrate that globotriaosylceramide is not a reliable marker to discriminate human centroblasts from centrocytes.	globotriaosylceramide	265-286	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	116-120
21788400-3	2011	We have previously shown that the cellular receptor for Shiga toxin B (STxB), the glycosphingolipid globotriaosylceramide (Gb(3) or CD77) is strongly increased in colorectal adenocarcinoma and their metastases.	globotriaosylceramide	100-121	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	132-136
21896204-1	2011	BACKGROUND: Anderson-Fabry disease (FD) is caused by a deficit of the alpha-galactosidase A enzyme which leads to the accumulation of complex sphingolipids, especially globotriaosylceramide (Gb3), in all the cells of the body, causing the onset of a multi-systemic disease with poor prognosis in adulthood.	globotriaosylceramide	168-189	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	191-194
21738355-1	2011	Fabry"s disease is an X-linked lysosomal storage disorder caused by abnormalities in the alpha-galactosidase A (GLA) gene, which leads to a GLA deficiency and to the intracellular deposition of globotriaosylceramide (Gb3) within vascular endothelium and other tissues.	globotriaosylceramide	194-215	galactosidase alpha	Homo sapiens	89-110
21738355-1	2011	Fabry"s disease is an X-linked lysosomal storage disorder caused by abnormalities in the alpha-galactosidase A (GLA) gene, which leads to a GLA deficiency and to the intracellular deposition of globotriaosylceramide (Gb3) within vascular endothelium and other tissues.	globotriaosylceramide	194-215	galactosidase alpha	Homo sapiens	112-115
21738355-1	2011	Fabry"s disease is an X-linked lysosomal storage disorder caused by abnormalities in the alpha-galactosidase A (GLA) gene, which leads to a GLA deficiency and to the intracellular deposition of globotriaosylceramide (Gb3) within vascular endothelium and other tissues.	globotriaosylceramide	194-215	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	217-220
21235448-2	2011	Absent or reduced enzyme activity leads to impaired catabolism of neutral glycosphingolipids, particularly globotriaosylceramide (Gb3), resulting in intracellular deposition of such lipids.	globotriaosylceramide	107-128	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	130-133
20131008-2	2011	The systemic accumulation of substrate, mainly globotriaosylceramide (Gb3), results in organ failure.	globotriaosylceramide	47-68	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	70-73
21477968-1	2011	BACKGROUND: Fabry disease results from deficiency of alpha-galactosidase A (AGA), causing lysosomal storage of globotriaosylceramide in heart and other tissues.	globotriaosylceramide	111-132	galactosidase, alpha	Mus musculus	53-74
21328014-1	2011	Fabry disease is a rare X-linked disorder caused by mutations in the alpha-galactosidase gene (GLA), the resultant deficiency of lysosomal alpha-galactosidase enzyme activity leading to systemic accumulation of globotriaosylceramide and other glycosphingolipids.	globotriaosylceramide	211-232	galactosidase, alpha	Mus musculus	95-98
21675497-1	2011	Fabry disease (FD) is an X-linked lysosomal disorder caused by the deficient activity of the enzyme alpha-galactosidase A, which leads to multisystemic storage of globotriaosylceramide in visceral tissues and vascular endothelium.	globotriaosylceramide	163-184	galactosidase alpha	Homo sapiens	100-121
21524384-3	2011	We have identified the P(k) blood group antigen (a GSL) globotriaosylceramide (Gb(3)) as a new resistance effector against HIV-1 infection.	globotriaosylceramide	56-77	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	79-84
21047542-1	2011	Fabry disease, due to the deficiency of alpha-galactosidase A (alpha-Gal), causes lysosomal accumulation of globotriaosylceramide (Gb3) in multiple tissues and prominently in the vascular endothelium.	globotriaosylceramide	108-129	galactosidase, alpha	Mus musculus	40-61
21047542-1	2011	Fabry disease, due to the deficiency of alpha-galactosidase A (alpha-Gal), causes lysosomal accumulation of globotriaosylceramide (Gb3) in multiple tissues and prominently in the vascular endothelium.	globotriaosylceramide	108-129	galactosidase, alpha	Mus musculus	63-72
21047542-1	2011	Fabry disease, due to the deficiency of alpha-galactosidase A (alpha-Gal), causes lysosomal accumulation of globotriaosylceramide (Gb3) in multiple tissues and prominently in the vascular endothelium.	globotriaosylceramide	131-134	galactosidase, alpha	Mus musculus	40-61
21047542-1	2011	Fabry disease, due to the deficiency of alpha-galactosidase A (alpha-Gal), causes lysosomal accumulation of globotriaosylceramide (Gb3) in multiple tissues and prominently in the vascular endothelium.	globotriaosylceramide	131-134	galactosidase, alpha	Mus musculus	63-72
20961863-0	2011	Increased globotriaosylceramide levels in a transgenic mouse expressing human alpha1,4-galactosyltransferase and a mouse model for treating Fabry disease.	globotriaosylceramide	10-31	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	78-108
20961863-1	2011	Fabry disease is a lysosomal storage disorder caused by an alpha-galactosidase A (alpha-Gal A) deficiency and resulting in the accumulation of glycosphingolipids, predominantly globotriaosylceramide (Gb3).	globotriaosylceramide	177-198	galactosidase, alpha	Mus musculus	82-93
20961863-1	2011	Fabry disease is a lysosomal storage disorder caused by an alpha-galactosidase A (alpha-Gal A) deficiency and resulting in the accumulation of glycosphingolipids, predominantly globotriaosylceramide (Gb3).	globotriaosylceramide	200-203	galactosidase, alpha	Mus musculus	59-80
20961863-1	2011	Fabry disease is a lysosomal storage disorder caused by an alpha-galactosidase A (alpha-Gal A) deficiency and resulting in the accumulation of glycosphingolipids, predominantly globotriaosylceramide (Gb3).	globotriaosylceramide	200-203	galactosidase, alpha	Mus musculus	82-93
20864368-1	2011	BACKGROUND: Fabry disease is caused by a deficiency of alpha-galactosidase A (alpha-Gal A), which results in the accumulation of globotriaosylceramide (GL3) and related glycosphingolipids in different organs.	globotriaosylceramide	129-150	galactosidase alpha	Homo sapiens	55-76
20846825-1	2011	In Fabry disease a deficiency of alpha-galactosidase A (alpha-gal A) activity leads to accumulation of globotriaosylceramide (Gb3) in various tissues including the heart.	globotriaosylceramide	103-124	galactosidase, alpha	Mus musculus	33-54
20846825-1	2011	In Fabry disease a deficiency of alpha-galactosidase A (alpha-gal A) activity leads to accumulation of globotriaosylceramide (Gb3) in various tissues including the heart.	globotriaosylceramide	103-124	galactosidase, alpha	Mus musculus	56-67
20846825-1	2011	In Fabry disease a deficiency of alpha-galactosidase A (alpha-gal A) activity leads to accumulation of globotriaosylceramide (Gb3) in various tissues including the heart.	globotriaosylceramide	126-129	galactosidase, alpha	Mus musculus	33-54
20846825-1	2011	In Fabry disease a deficiency of alpha-galactosidase A (alpha-gal A) activity leads to accumulation of globotriaosylceramide (Gb3) in various tissues including the heart.	globotriaosylceramide	126-129	galactosidase, alpha	Mus musculus	56-67
20941593-1	2011	Fabry disease is a multisystem X-linked disorder resulting from alpha-galactosidase A (alpha-GalA) gene mutations leading to the accumulation of globotriaosylceramide mainly in endothelium compromising heart, kidney, and brain.	globotriaosylceramide	145-166	galactosidase alpha	Homo sapiens	64-85
20941593-1	2011	Fabry disease is a multisystem X-linked disorder resulting from alpha-galactosidase A (alpha-GalA) gene mutations leading to the accumulation of globotriaosylceramide mainly in endothelium compromising heart, kidney, and brain.	globotriaosylceramide	145-166	galactosidase alpha	Homo sapiens	87-97
23430826-1	2011	Fabry disease is an X-linked glycosphingolipidosis caused by deficient synthesis of the enzyme alpha-galactosidase A, which results in glycosphingolipidosis, predominantly globotriaosylceramide, progressively accumulating in systemic tissue.	globotriaosylceramide	172-193	galactosidase alpha	Homo sapiens	95-116
21255370-3	2011	Stx binds specifically to the glycosphingolipid globotriaosylceramide (Gb3) at the surface of target cells and is then internalized by endocytosis.	globotriaosylceramide	48-69	ST8 alpha-N-acetyl-neuraminide alpha-2,8-sialyltransferase 2	Homo sapiens	0-3
21255370-3	2011	Stx binds specifically to the glycosphingolipid globotriaosylceramide (Gb3) at the surface of target cells and is then internalized by endocytosis.	globotriaosylceramide	48-69	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	71-74
21428036-2	2011	Deficiency of alpha-galactosidase A activity leads to the accumulation of neutral glycosphingolipids, primarily globotriaosylceramide (GL-3) in various tissues, particularly blood vessels, kidneys, myocardium and in ganglions of the peripheral and autonomic nervous system and causes diverse symptoms.	globotriaosylceramide	112-133	galactosidase alpha	Homo sapiens	14-35
19919901-3	2011	There is an inability to catabolize lipids which results in cellular accumulation of its most abundant substrate, globotriaosylceramide (Gb3), and other neutral glycosphingolipids in vascular endothelium and other tissues throughout the body.	globotriaosylceramide	114-135	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	137-140
20864368-1	2011	BACKGROUND: Fabry disease is caused by a deficiency of alpha-galactosidase A (alpha-Gal A), which results in the accumulation of globotriaosylceramide (GL3) and related glycosphingolipids in different organs.	globotriaosylceramide	129-150	galactosidase alpha	Homo sapiens	78-89
21331308-1	2010	Fabry disease is a rare X-linked lysosomal storage disorder caused by a deficiency in alpha-galactosidase A (GLA), which catalyzes the hydrolysis of terminal alpha-galactosyl groups from glycosphingolipids, such as globotriaosylceramide (Gb3).	globotriaosylceramide	215-236	galactosidase alpha	Homo sapiens	86-107
21331308-1	2010	Fabry disease is a rare X-linked lysosomal storage disorder caused by a deficiency in alpha-galactosidase A (GLA), which catalyzes the hydrolysis of terminal alpha-galactosyl groups from glycosphingolipids, such as globotriaosylceramide (Gb3).	globotriaosylceramide	215-236	galactosidase alpha	Homo sapiens	109-112
20716521-3	2010	To evaluate this relationship, Verotoxin binding its receptor GSL, globotriaosyl ceramide (Gb(3)), was analyzed in simple GSL/cholesterol, detergent-resistant membrane vesicles by equilibrium density gradient centrifugation.	globotriaosylceramide	67-89	cathepsin A	Homo sapiens	62-65
21211678-2	2010	In males, Gb(3) measurement allows to confirm the diagnosis which is based on deficient alpha-galactosidase A (alpha-Gal A) activity in leukocytes.	globotriaosylceramide	10-15	galactosidase alpha	Homo sapiens	88-109
21211678-2	2010	In males, Gb(3) measurement allows to confirm the diagnosis which is based on deficient alpha-galactosidase A (alpha-Gal A) activity in leukocytes.	globotriaosylceramide	10-15	galactosidase alpha	Homo sapiens	111-122
21211678-7	2010	Our study shows that urinary Gb(3) (measurement and C24/C18 ratio) allows the diagnosis of 92 % of classical FD heterozygotes, and is often normal in variant FD heterozygotes.	globotriaosylceramide	29-34	Bardet-Biedl syndrome 9	Homo sapiens	56-59
21092187-4	2010	Deficient activity of lysosomal alpha-galactosidase A results in progressive accumulation of globotriaosylceramide within lysosomes, believed to trigger a cascade of cellular events.	globotriaosylceramide	93-114	galactosidase alpha	Homo sapiens	32-53
21088081-1	2010	Anderson-Fabry disease is an X-linked lysosomal storage disorder resulting from a deficiency of the enzyme alpha-galactosidase A (alpha-Gal A) and subsequent cellular storage of the enzyme"s substrate globotriaosylceramide (Gb3) and related glycosphingolipids.	globotriaosylceramide	201-222	galactosidase alpha	Homo sapiens	107-128
21088081-1	2010	Anderson-Fabry disease is an X-linked lysosomal storage disorder resulting from a deficiency of the enzyme alpha-galactosidase A (alpha-Gal A) and subsequent cellular storage of the enzyme"s substrate globotriaosylceramide (Gb3) and related glycosphingolipids.	globotriaosylceramide	201-222	galactosidase alpha	Homo sapiens	130-141
21088081-1	2010	Anderson-Fabry disease is an X-linked lysosomal storage disorder resulting from a deficiency of the enzyme alpha-galactosidase A (alpha-Gal A) and subsequent cellular storage of the enzyme"s substrate globotriaosylceramide (Gb3) and related glycosphingolipids.	globotriaosylceramide	201-222	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	224-227
20852936-1	2010	Fabry"s disease is an X-linked recessive disorder that results from the deficiency of alpha-galactosidase A and causes the accumulation of globotriaosylceramide (Gb3) in different tissues.	globotriaosylceramide	139-160	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	162-165
20837469-1	2010	Globotriaosylceramide (Gb3) is a well known receptor for Shiga toxin (Stx), produced by enterohemorrhagic Escherichia coli and Shigella dysenteriae.	globotriaosylceramide	0-21	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	23-26
20644116-4	2010	Stx occurs in 2 variants, Stx1 and Stx2, each of which is composed of 1 catalytically active A subunit that is responsible for cytotoxicity, and 5 identical B subunits that mediate binding to cell-surface globo-triaosylceramide.	globotriaosylceramide	205-227	syntaxin 2	Homo sapiens	35-39
20644116-6	2010	This rapid effect requires binding and clustering of globotriaosylceramide, and depends on plasma membrane cholesterol and caveolin-1 but not clathrin.	globotriaosylceramide	53-74	caveolin 1	Homo sapiens	123-133
21170881-2	2010	The resulting deficiency in a-GalA activity leads to intra-lysosomal accumulation of neutral glycosphingolipids, mainly globotriaosylceramide (Gb3), in various organ systems.	globotriaosylceramide	120-141	galactosidase alpha	Homo sapiens	30-34
21170881-2	2010	The resulting deficiency in a-GalA activity leads to intra-lysosomal accumulation of neutral glycosphingolipids, mainly globotriaosylceramide (Gb3), in various organ systems.	globotriaosylceramide	120-141	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	143-146
20716521-3	2010	To evaluate this relationship, Verotoxin binding its receptor GSL, globotriaosyl ceramide (Gb(3)), was analyzed in simple GSL/cholesterol, detergent-resistant membrane vesicles by equilibrium density gradient centrifugation.	globotriaosylceramide	91-96	cathepsin A	Homo sapiens	62-65
20716521-10	2010	We found GalCer and GlcCer bind Gb(3), suggesting GSL-GSL interaction can counter cholesterol masking of Gb(3).	globotriaosylceramide	32-37	cathepsin A	Homo sapiens	50-53
20716521-10	2010	We found GalCer and GlcCer bind Gb(3), suggesting GSL-GSL interaction can counter cholesterol masking of Gb(3).	globotriaosylceramide	32-37	cathepsin A	Homo sapiens	54-57
20716521-10	2010	We found GalCer and GlcCer bind Gb(3), suggesting GSL-GSL interaction can counter cholesterol masking of Gb(3).	globotriaosylceramide	105-110	cathepsin A	Homo sapiens	50-53
20716521-10	2010	We found GalCer and GlcCer bind Gb(3), suggesting GSL-GSL interaction can counter cholesterol masking of Gb(3).	globotriaosylceramide	105-110	cathepsin A	Homo sapiens	54-57
19515805-1	2009	BACKGROUND: In Fabry disease, storage of globotriaosylceramide (Gb3) in arterial walls is one of the main pathogenetic factors that are thought to underlie the clinical manifestations of the disease.	globotriaosylceramide	41-62	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	64-67
20471476-1	2010	Fabry disease is an X-linked lysosomal storage disorder due to deficiency of alpha-Galactosidase A, causing accumulation of globotriaosylceramide and elevated plasma globotriaosylsphingosine (lysoGb3).	globotriaosylceramide	124-145	galactosidase alpha	Homo sapiens	77-98
19631563-2	2010	Treatment with recombinant enzyme preparations aims to attenuate and reverse accumulation of the major enzyme substrate, globotriaosylceramide (Gb3).	globotriaosylceramide	121-142	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	144-147
19948172-1	2010	The glycosphingolipid globotriaosyl ceramide, (Galalpha1-4Galss1-4 glucosyl ceramide-Gb(3)) also known as CD77 and the P(k) blood group antigen, is bound by both verotoxins and by the HIV adhesin, gp120.	globotriaosylceramide	22-44	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	106-110
19948172-1	2010	The glycosphingolipid globotriaosyl ceramide, (Galalpha1-4Galss1-4 glucosyl ceramide-Gb(3)) also known as CD77 and the P(k) blood group antigen, is bound by both verotoxins and by the HIV adhesin, gp120.	globotriaosylceramide	22-44	inter-alpha-trypsin inhibitor heavy chain 4	Homo sapiens	197-202
19948172-5	2010	Differential binding of verotoxins and gp120 to such Gb(3) isoforms in model and cell membranes indicates a significant role in the eventual pathogenic outcome.	globotriaosylceramide	53-58	inter-alpha-trypsin inhibitor heavy chain 4	Homo sapiens	39-44
20092352-5	2010	Stx1 preferred the Pk trisaccharide of its native receptor, globotriaosylceramide (Gb3), while the more potent and clinically relevant variant, Stx2, preferred the Pk trisaccharide with the terminal galactose replaced with N-acetylgalactosamine (NHAc-Pk).	globotriaosylceramide	60-81	syntaxin 1A	Homo sapiens	0-4
19895884-1	2010	Verotoxin (VT-1) is a cytotoxin, produced by Shigella dysenteriae type 1 or by Shiga toxin-producing Escherichia coli, which binds specifically to globotriaosylceramide (Gb3).	globotriaosylceramide	147-168	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	170-173
20228022-1	2010	Fabry disease is an X- linked lysosomal disorder due to deficient activity of the enzyme alpha galactosidase A which leads to multisystemic storage of globotriaosylceramide with neurologic, gastrointestinal, cardiac, renal, skin and ophtalmological involvement.	globotriaosylceramide	151-172	galactosidase alpha	Homo sapiens	89-110
19242721-2	2009	In affected patients, the enzyme substrate, globotriaosylceramide (Gb3), accumulates in cells of various tissues and organs.	globotriaosylceramide	44-65	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	67-70
19859978-1	2009	Fabry disease is an X-linked lysosomal disorder that results from a deficiency of the lysosomal enzyme alpha-galactosidase A leading to accumulation of glycolipids, mainly globotriaosylceramide in the cells from different tissues.	globotriaosylceramide	172-193	galactosidase alpha	Homo sapiens	103-124
19818152-1	2009	BACKGROUND: In Fabry disease (alpha-galactosidase A deficiency) accumulation of Globotriaosylceramide (Gb3) leads to progressive organ failure and premature death.	globotriaosylceramide	80-101	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	103-106
20660166-1	2010	Fabry disease is an X-linked inherited condition due to the absence or reduction of alpha-galactosidase activity in lysosomes, that results in accumulation of globotriaosylceramide (Gb3) and related neutral glycosphingolipids.	globotriaosylceramide	159-180	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	182-185
20347160-2	2010	In this work intracerebroventricular administration of Stx2 in rat brains significantly increased the expression of its receptor globotriaosylceramide (Gb(3)) in neuronal populations from striatum, hippocampus and cortex.	globotriaosylceramide	129-150	syntaxin 2	Rattus norvegicus	55-59
20347160-2	2010	In this work intracerebroventricular administration of Stx2 in rat brains significantly increased the expression of its receptor globotriaosylceramide (Gb(3)) in neuronal populations from striatum, hippocampus and cortex.	globotriaosylceramide	152-158	syntaxin 2	Rattus norvegicus	55-59
20347160-3	2010	Stx2 was immunodetected in neurons that expressed Gb(3) after intracerebroventricular administration of the toxin.	globotriaosylceramide	50-55	syntaxin 2	Rattus norvegicus	0-4
20347160-4	2010	Confocal immunofluorescence of microtubule-associated protein 2 showed aberrant dendrites in neurons expressing increased Gb(3).	globotriaosylceramide	122-127	microtubule-associated protein 2	Rattus norvegicus	31-63
20347160-8	2010	We thus suggest that Stx2 induces the expression of Gb(3) in neurons and triggers neuronal dysfunctions.	globotriaosylceramide	52-57	syntaxin 2	Rattus norvegicus	21-25
19965549-2	2010	Traditionally, globotriaosylceramide (Gb(3)) in urine has been used to evaluate the effect of specific therapy, such as enzyme replacement therapy (ERT).	globotriaosylceramide	15-36	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	38-43
20345350-2	2010	This leads to a progressive accumulation of globotriaosylceramide (Gb3) in the lysosomes of different cells and tissues, causing principally ventricular hypertrophy, renal failure and cerebrovascular accidents, reducing lifespan both in hemizygous males and heterozygous females.	globotriaosylceramide	44-65	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	67-70
19853240-5	2009	The enzyme cleaved globotriaosylceramide (Gb3) accumulated in cultured fibroblasts from a patient with Fabry disease.	globotriaosylceramide	19-40	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	42-45
19674101-1	2009	Abnormal biosynthesis of globotriaosylceramide (Gb3) is known to be associated with Gb3-related diseases, such as Fabry disease.	globotriaosylceramide	25-46	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	48-51
19674101-1	2009	Abnormal biosynthesis of globotriaosylceramide (Gb3) is known to be associated with Gb3-related diseases, such as Fabry disease.	globotriaosylceramide	25-46	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	84-87
19639234-1	2009	Fabry disease is a lysosomal storage disease caused by a deficiency of alpha-galactosidase A, which results in aberrant glycosphingolipid metabolism and accumulation of globotriaosylceramide (Gb3).	globotriaosylceramide	169-190	galactosidase, alpha	Mus musculus	71-92
19639234-1	2009	Fabry disease is a lysosomal storage disease caused by a deficiency of alpha-galactosidase A, which results in aberrant glycosphingolipid metabolism and accumulation of globotriaosylceramide (Gb3).	globotriaosylceramide	192-195	galactosidase, alpha	Mus musculus	71-92
18716315-1	2009	Glycosphingolipid (GSL) fatty acid strictly regulates verotoxin 1 (VT1) and the HIV adhesin, gp120 binding to globotriaosyl ceramide within Gb(3)/cholesterol detergent resistant membrane (DRM) vesicle constructs and in Gb(3) water-air interface monolayers in a similar manner.	globotriaosylceramide	110-132	inter-alpha-trypsin inhibitor heavy chain 4	Homo sapiens	93-98
19628671-5	2009	Treatment of knockout mice with a potent inhibitor of glucosylceramide synthase reversed accumulation of globotriaosylceramide but failed to normalize the defect in vasorelaxation.	globotriaosylceramide	105-126	UDP-glucose ceramide glucosyltransferase	Mus musculus	54-79
18716315-1	2009	Glycosphingolipid (GSL) fatty acid strictly regulates verotoxin 1 (VT1) and the HIV adhesin, gp120 binding to globotriaosyl ceramide within Gb(3)/cholesterol detergent resistant membrane (DRM) vesicle constructs and in Gb(3) water-air interface monolayers in a similar manner.	globotriaosylceramide	140-145	inter-alpha-trypsin inhibitor heavy chain 4	Homo sapiens	93-98
18716315-1	2009	Glycosphingolipid (GSL) fatty acid strictly regulates verotoxin 1 (VT1) and the HIV adhesin, gp120 binding to globotriaosyl ceramide within Gb(3)/cholesterol detergent resistant membrane (DRM) vesicle constructs and in Gb(3) water-air interface monolayers in a similar manner.	globotriaosylceramide	219-224	inter-alpha-trypsin inhibitor heavy chain 4	Homo sapiens	93-98
19714252-3	2009	We determined the expression of globotriaosylceramide (Gb3Cer/CD77), the Shiga toxin receptor, in human pancreatic and colon adenocarcinomas.	globotriaosylceramide	32-53	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	62-66
19495571-1	2009	UNLABELLED: Fabry disease is a rare, X-linked lysosomal storage disease caused by an inborn deficiency of alpha-galactosidase A, which results in the progressive accumulation of globotriaosylceramide and other neutral glycolipids in a range of cells and tissues.	globotriaosylceramide	178-199	galactosidase alpha	Homo sapiens	106-127
19464216-1	2009	Fabry disease is an X-linked lysosomal storage disorder caused by deficiency of alpha-galactosidase A, resulting in accumulation of the principal substrate, globotriaosylceramide (Gb(3)), in various physiological fluids and tissues in affected patients.	globotriaosylceramide	157-178	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	180-185
19444268-4	2009	They also propose that the binding of the HIV-1 glycoprotein gp120 to Gb(3) in renal tubules may play a role in HIV nephropathy.	globotriaosylceramide	70-75	Envelope surface glycoprotein gp160, precursor	Human immunodeficiency virus 1	61-66
19387866-1	2009	Fabry disease is an X-linked lysosomal storage disorder caused by mutations in the gene encoding alpha-galactosidase A (alpha-Gal A), with consequent accumulation of its major glycosphingolipid substrate, globotriaosylceramide (GL-3).	globotriaosylceramide	205-226	galactosidase alpha	Homo sapiens	97-118
19387866-1	2009	Fabry disease is an X-linked lysosomal storage disorder caused by mutations in the gene encoding alpha-galactosidase A (alpha-Gal A), with consequent accumulation of its major glycosphingolipid substrate, globotriaosylceramide (GL-3).	globotriaosylceramide	205-226	galactosidase alpha	Homo sapiens	120-131
19268437-0	2009	An easy and sensitive method for determination of globotriaosylceramide (Gb3) from urinary sediment: utility for Fabry disease diagnosis and treatment monitoring.	globotriaosylceramide	50-71	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	73-76
19470247-0	2009	Globotriaosylceramide (Gb3) content in HeLa cells is correlated to Shiga toxin-induced cytotoxicity and Gb3 synthase expression.	globotriaosylceramide	0-21	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	104-116
19470247-0	2009	Globotriaosylceramide (Gb3) content in HeLa cells is correlated to Shiga toxin-induced cytotoxicity and Gb3 synthase expression.	globotriaosylceramide	23-26	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	104-116
19268437-2	2009	The defect leads to the accumulation of globotriaosylceramide (Gb3).	globotriaosylceramide	40-61	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	63-66
19265719-1	2009	PURPOSE: Fabry disease, a genetic deficiency of alpha-galactosidase A, is characterized by pathogenic cellular accumulation of globotriaosylceramide.	globotriaosylceramide	127-148	galactosidase alpha	Homo sapiens	48-69
19202000-1	2009	The lysosomal storage disorder Fabry disease is characterized by excessive globotriaosylceramide (Gb3) accumulation in major organs such as the heart and kidney.	globotriaosylceramide	75-96	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	98-101
19243984-0	2009	Falsely elevated urinary Gb3 (globotriaosylceramide, CTH, GL3).	globotriaosylceramide	30-51	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	25-28
19265719-2	2009	During clinical trials, recombinant human alpha-galactosidase A (agalsidase beta; Fabrazyme, Genzyme Corporation, Cambridge, MA), infused intravenously at 1.0 mg/kg every 2 weeks for 6 months, cleared or reduced globotriaosylceramide in renal, cardiac, and dermal microvascular endothelia and other cells, with results sustained for up to 5 years in most patients evaluated.	globotriaosylceramide	212-233	galactosidase alpha	Homo sapiens	42-63
19169844-1	2009	Fabry disease is a complex, multisystemic and clinically heterogeneous disease with prominent urinary excretion of globotriaosylceramide (Gb(3)), the principal substrate of the deficient enzyme, alpha-galactosidase A.	globotriaosylceramide	115-136	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	138-143
19169844-1	2009	Fabry disease is a complex, multisystemic and clinically heterogeneous disease with prominent urinary excretion of globotriaosylceramide (Gb(3)), the principal substrate of the deficient enzyme, alpha-galactosidase A.	globotriaosylceramide	115-136	galactosidase alpha	Homo sapiens	195-216
19180148-2	2009	Increased membrane globotriaosylceramide (Gb3/CD77) expression was observed; it is suggested that this finding may be potentially useful as a surrogate marker of disease severity.	globotriaosylceramide	19-40	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	42-45
19282651-3	2009	Fabry"s disease is an X-linked lysosomal storage disorder caused by a deficiency of the enzyme alpha-galactosidase A, which results in progressive intracellular accumulation of globotriaosylceramide (Gb3) in various organs including the heart.	globotriaosylceramide	177-198	galactosidase alpha	Homo sapiens	95-116
19282651-3	2009	Fabry"s disease is an X-linked lysosomal storage disorder caused by a deficiency of the enzyme alpha-galactosidase A, which results in progressive intracellular accumulation of globotriaosylceramide (Gb3) in various organs including the heart.	globotriaosylceramide	177-198	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	200-203
19013219-3	2009	From the results of immunocytochemical analysis, both HSP70 and HSC70 were constitutively expressed in globotriaosylceramide (Gb3)-expressing Raji cells as well as Gb3-negative K562 cells.	globotriaosylceramide	103-124	heat shock protein family A (Hsp70) member 4	Homo sapiens	54-59
19013219-3	2009	From the results of immunocytochemical analysis, both HSP70 and HSC70 were constitutively expressed in globotriaosylceramide (Gb3)-expressing Raji cells as well as Gb3-negative K562 cells.	globotriaosylceramide	103-124	heat shock protein family A (Hsp70) member 8	Homo sapiens	64-69
19013219-3	2009	From the results of immunocytochemical analysis, both HSP70 and HSC70 were constitutively expressed in globotriaosylceramide (Gb3)-expressing Raji cells as well as Gb3-negative K562 cells.	globotriaosylceramide	103-124	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	126-129
19253940-7	2009	The strategy for the synthesis employed a Gb(3)-MUC5AC thioester cassette as a key building block.	globotriaosylceramide	42-47	mucin 5AC, oligomeric mucus/gel-forming	Homo sapiens	48-54
19037253-1	2009	Anderson-Fabry disease, an inherited deficiency in the lysosomal enzyme alpha-galactosidase A, is characterized by the progressive accumulation of globotriaosylceramide (Gb3), also known as CD77.	globotriaosylceramide	147-168	galactosidase alpha	Homo sapiens	72-93
19037253-1	2009	Anderson-Fabry disease, an inherited deficiency in the lysosomal enzyme alpha-galactosidase A, is characterized by the progressive accumulation of globotriaosylceramide (Gb3), also known as CD77.	globotriaosylceramide	147-168	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	170-173
19037253-1	2009	Anderson-Fabry disease, an inherited deficiency in the lysosomal enzyme alpha-galactosidase A, is characterized by the progressive accumulation of globotriaosylceramide (Gb3), also known as CD77.	globotriaosylceramide	147-168	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	190-194
19180148-2	2009	Increased membrane globotriaosylceramide (Gb3/CD77) expression was observed; it is suggested that this finding may be potentially useful as a surrogate marker of disease severity.	globotriaosylceramide	19-40	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	46-50
18451534-1	2008	Silurus asotus lectin (SAL) is a member of the rhamnose-binding lectin (RBL) family, and recognizes globotriaosylceramide (Gb3) on the cell surface of Burkitt"s lymphoma cell lines, such as Raji and Daudi cells.	globotriaosylceramide	100-121	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	123-126
19190807-2	2009	Binding of Stx to the glycosphingolipid (GSL) globotriaosylceramide (Gb3Cer/CD77) on endothelial cells followed by receptor-mediated endocytosis is the linchpin in STEC-mediated disease.	globotriaosylceramide	46-67	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	76-80
18707907-1	2008	Fabry disease, an X-linked systemic vasculopathy, is caused by a deficiency of alpha-galactosidase A resulting in globotriaosylceramide (Gb(3)) storage in cells.	globotriaosylceramide	114-135	galactosidase alpha	Homo sapiens	79-100
18707907-1	2008	Fabry disease, an X-linked systemic vasculopathy, is caused by a deficiency of alpha-galactosidase A resulting in globotriaosylceramide (Gb(3)) storage in cells.	globotriaosylceramide	137-143	galactosidase alpha	Homo sapiens	79-100
18707907-5	2008	Increased Gb(3) also induced expression of intercellular adhesion molecule-1, vascular cell adhesion molecule-1, and E-selectin.	globotriaosylceramide	10-15	intercellular adhesion molecule 1	Homo sapiens	43-76
18707907-5	2008	Increased Gb(3) also induced expression of intercellular adhesion molecule-1, vascular cell adhesion molecule-1, and E-selectin.	globotriaosylceramide	10-15	vascular cell adhesion molecule 1	Homo sapiens	78-111
18707907-5	2008	Increased Gb(3) also induced expression of intercellular adhesion molecule-1, vascular cell adhesion molecule-1, and E-selectin.	globotriaosylceramide	10-15	selectin E	Homo sapiens	117-127
18707907-6	2008	Reduction of endogenous Gb(3) by treatment of the cells with an inhibitor of glycosphingolipid synthase or alpha-galactosidase A led to decreased expression of adhesion molecules.	globotriaosylceramide	24-29	galactosidase alpha	Homo sapiens	107-128
18564063-2	2008	STxB (Shiga toxin B-subunit) is known to bind the glycosphingolipid Gb3 (globotriaosyl ceramide), which is overexpressed by various human tumours.	globotriaosylceramide	73-95	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	68-71
18757779-2	2008	Several studies have demonstrated that VT induces endothelial cell (EC) death via the VT receptor globotriaosylceramide (Gb3/CD77) leading to this symptom.	globotriaosylceramide	98-119	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	121-124
18757779-2	2008	Several studies have demonstrated that VT induces endothelial cell (EC) death via the VT receptor globotriaosylceramide (Gb3/CD77) leading to this symptom.	globotriaosylceramide	98-119	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	125-129
18754742-2	2008	Globotriaosylceramide (Gb(3)), the receptor for Stx2, was localized to neurons of the central nervous system (CNS) of normal mice.	globotriaosylceramide	0-21	syntaxin 2	Mus musculus	48-52
18754742-2	2008	Globotriaosylceramide (Gb(3)), the receptor for Stx2, was localized to neurons of the central nervous system (CNS) of normal mice.	globotriaosylceramide	23-29	syntaxin 2	Mus musculus	48-52
18565198-2	2008	Defective Gla results in multi-organ accumulation of neutral glycosphingolipids (GSLs), especially in the vascular endothelium, with the major GSL accumulated being globotriaosylceramide (Gb3).	globotriaosylceramide	165-186	galactosidase, alpha	Mus musculus	10-13
18565198-2	2008	Defective Gla results in multi-organ accumulation of neutral glycosphingolipids (GSLs), especially in the vascular endothelium, with the major GSL accumulated being globotriaosylceramide (Gb3).	globotriaosylceramide	188-191	galactosidase, alpha	Mus musculus	10-13
18522550-2	2008	In absence of enzyme replacement therapy (ERT), globotriaosylceramide (Gb3) accumulates in tissue, leading to progressive organ damage with severe renal, cardiac and central nervous system complications.	globotriaosylceramide	48-69	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	71-74
18339187-2	2008	The subsequent accumulation of globotriaosylceramide (Gb3) in cells and tissues of the body has multisystemic effects and significantly impacts upon quality of life and survival of individuals with this condition.	globotriaosylceramide	31-52	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	54-57
18297328-1	2008	UNLABELLED: Fabry disease is an X-linked lysosomal deficiency of alpha-galactosidase A that results in cellular accumulation of galactoconjugates, mainly globotriaosylceramide, particularly in blood vessels.	globotriaosylceramide	154-175	galactosidase alpha	Homo sapiens	65-86
18219591-0	2008	Naked plasmid DNA-based alpha-galactosidase A gene transfer partially reduces systemic accumulation of globotriaosylceramide in Fabry mice.	globotriaosylceramide	103-124	galactosidase, alpha	Mus musculus	24-45
18048405-4	2008	Globotriaosylceramide (Gb3) exists as a natural isomer for iGb3, and both isomers are frequently found together in mixtures of glycosphingolipids extracted from mammalian cell membranes.	globotriaosylceramide	0-21	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	23-26
18086809-7	2008	Additionally, SB202190 reduced the cellular globotriaosylceramide content under LPS- and TNF-alpha-induced conditions.	globotriaosylceramide	44-65	tumor necrosis factor	Homo sapiens	89-98
18023222-1	2008	Fabry disease is a complex, multisystemic and clinically heterogeneous disease, in which the urinary excretion of globotriaosylceramide (Gb3), the principal substrate of the deficient enzyme, alpha-galactosidase A, is more prominent than the increased concentrations of the lipid in the plasma of affected hemizygotes and heterozygotes.	globotriaosylceramide	114-135	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	137-140
18023222-1	2008	Fabry disease is a complex, multisystemic and clinically heterogeneous disease, in which the urinary excretion of globotriaosylceramide (Gb3), the principal substrate of the deficient enzyme, alpha-galactosidase A, is more prominent than the increased concentrations of the lipid in the plasma of affected hemizygotes and heterozygotes.	globotriaosylceramide	114-135	galactosidase alpha	Homo sapiens	192-213
18219591-1	2008	Fabry disease is an X-linked recessive inborn metabolic disorder in which a deficiency in lysosomal enzyme alpha-galactosidase A (Gal A) causes the systemic accumulation of globotriaosylceramide (Gb3).	globotriaosylceramide	173-194	galactosidase, alpha	Mus musculus	107-128
18219591-1	2008	Fabry disease is an X-linked recessive inborn metabolic disorder in which a deficiency in lysosomal enzyme alpha-galactosidase A (Gal A) causes the systemic accumulation of globotriaosylceramide (Gb3).	globotriaosylceramide	173-194	galactosidase, alpha	Mus musculus	130-135
18219591-1	2008	Fabry disease is an X-linked recessive inborn metabolic disorder in which a deficiency in lysosomal enzyme alpha-galactosidase A (Gal A) causes the systemic accumulation of globotriaosylceramide (Gb3).	globotriaosylceramide	196-199	galactosidase, alpha	Mus musculus	107-128
18219591-1	2008	Fabry disease is an X-linked recessive inborn metabolic disorder in which a deficiency in lysosomal enzyme alpha-galactosidase A (Gal A) causes the systemic accumulation of globotriaosylceramide (Gb3).	globotriaosylceramide	196-199	galactosidase, alpha	Mus musculus	130-135
17940933-4	2007	The enzyme deficiency leads to a progressive accumulation of globotriaosylceramide (Gb3) in various tissues and organ systems and is responsible for the large variability of the clinical signs and symptoms of the disease.	globotriaosylceramide	61-82	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	84-87
17965340-3	2007	It is demonstrated in this study that Stx2 binds to human neutrophils by a non-classical mechanism that is independent of Gb(3).	globotriaosylceramide	122-127	syntaxin 2	Homo sapiens	38-42
17965340-4	2007	In contrast, the investigation revealed that Stx2 binds to murine neutrophils by the classical Gb(3)-dependent mechanism.	globotriaosylceramide	95-100	syntaxin 2	Mus musculus	45-49
17849232-1	2007	Fabry disease, or alpha-galactosidase A (alpha-Gal A) deficiency, is a lysosomal storage disorder in which accumulation of globotriaosylceramide (Gb(3)) is thought to be responsible for the development of renal, cardiac and cerebral complications.	globotriaosylceramide	123-144	galactosidase alpha	Homo sapiens	18-39
17849232-1	2007	Fabry disease, or alpha-galactosidase A (alpha-Gal A) deficiency, is a lysosomal storage disorder in which accumulation of globotriaosylceramide (Gb(3)) is thought to be responsible for the development of renal, cardiac and cerebral complications.	globotriaosylceramide	123-144	galactosidase alpha	Homo sapiens	41-52
17849232-1	2007	Fabry disease, or alpha-galactosidase A (alpha-Gal A) deficiency, is a lysosomal storage disorder in which accumulation of globotriaosylceramide (Gb(3)) is thought to be responsible for the development of renal, cardiac and cerebral complications.	globotriaosylceramide	146-151	galactosidase alpha	Homo sapiens	18-39
17849232-1	2007	Fabry disease, or alpha-galactosidase A (alpha-Gal A) deficiency, is a lysosomal storage disorder in which accumulation of globotriaosylceramide (Gb(3)) is thought to be responsible for the development of renal, cardiac and cerebral complications.	globotriaosylceramide	146-151	galactosidase alpha	Homo sapiens	41-52
18022018-1	2007	Fabry disease, an X-linked recessive glycolipid storage disease, is caused by a deficiency of the lysosomal enzyme alpha-galactosidase A (alpha-Gal A), which cleaves a fatty substance called globotriaosylceramide (GL3).	globotriaosylceramide	191-212	galactosidase alpha	Homo sapiens	115-136
18022018-1	2007	Fabry disease, an X-linked recessive glycolipid storage disease, is caused by a deficiency of the lysosomal enzyme alpha-galactosidase A (alpha-Gal A), which cleaves a fatty substance called globotriaosylceramide (GL3).	globotriaosylceramide	191-212	galactosidase alpha	Homo sapiens	138-149
17570073-9	2007	Over the years, we have established a dynamic process, developing techniques or new reagents to detect as many treatable disorders as possible, now evaluating macromolecules associated with lysosomal storage disorders, mainly globotriaosylceramide (Gb3) for Fabry disease.	globotriaosylceramide	226-247	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	249-252
17697536-1	2007	Fabry"s disease, a disorder affecting the gene for the lysosomal enzyme alpha-galactosidase A (alpha-GAL A), can cause accumulation of globotriaosylceramide (GL-3) in the vascular endothelial cells.	globotriaosylceramide	135-156	galactosidase, alpha	Mus musculus	72-93
17535804-0	2007	Caveolin-associated accumulation of globotriaosylceramide in the vascular endothelium of alpha-galactosidase A null mice.	globotriaosylceramide	36-57	galactosidase, alpha	Mus musculus	89-110
17535804-2	2007	The enzymatic defect results in the deposition of globotriaosylceramide (Gb3) in the vascular endothelium.	globotriaosylceramide	50-71	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	73-76
17697536-1	2007	Fabry"s disease, a disorder affecting the gene for the lysosomal enzyme alpha-galactosidase A (alpha-GAL A), can cause accumulation of globotriaosylceramide (GL-3) in the vascular endothelial cells.	globotriaosylceramide	135-156	galactosidase, alpha	Mus musculus	95-106
17395657-1	2007	BACKGROUND: Fabry disease (FD) is caused by an X-linked deficiency in the activity of alpha-galactosidase A and the resultant accumulation of globotriaosylceramide (Gb3) in multiple tissues.	globotriaosylceramide	142-163	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	165-168
17409312-1	2007	Fabry disease, an inherited deficiency of the lysosomal enzyme alpha-galactosidase A, causes progressive intralysosomal accumulation of globotriaosylceramide (GL-3) and premature death from renal, cardiac, and cerebrovascular manifestations.	globotriaosylceramide	136-157	galactosidase alpha	Homo sapiens	63-84
17160925-2	2006	DEVELOPMENT: Fabry disease is a hereditary deficiency of lisosomal alpha-galactosidase A resulting in accumulation of globotriaosylceramide in vascular endothelium and smooth-muscle cells.	globotriaosylceramide	118-139	galactosidase alpha	Homo sapiens	67-88
17336267-3	2007	Engagement of Gb(3)/CD77 with SLT-B reduced the amount of CD20 and CXCR4 available, but levels of BCR, MHC Class II, CD21, CD27 and CD54 remained unchanged.	globotriaosylceramide	14-19	keratin 20	Homo sapiens	58-62
17336267-3	2007	Engagement of Gb(3)/CD77 with SLT-B reduced the amount of CD20 and CXCR4 available, but levels of BCR, MHC Class II, CD21, CD27 and CD54 remained unchanged.	globotriaosylceramide	14-19	C-X-C motif chemokine receptor 4	Homo sapiens	67-72
17336267-3	2007	Engagement of Gb(3)/CD77 with SLT-B reduced the amount of CD20 and CXCR4 available, but levels of BCR, MHC Class II, CD21, CD27 and CD54 remained unchanged.	globotriaosylceramide	14-19	CD27 molecule	Homo sapiens	123-127
17409683-0	2007	[Regulation of globotriaosylceramide (Gb3)-mediated signal transduction by rhamnose-binding lectin].	globotriaosylceramide	15-36	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	38-41
17409683-1	2007	Silurus asotus (catfish) egg lectin (SAL) has potent affinity to Gal alpha-linked carbohydrate chains of not only glycoproteins but also glycosphingolipids such as globotriaosylceramide (Gb3).	globotriaosylceramide	164-185	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	187-190
17171433-1	2007	Fabry disease is an X-linked lysosomal storage disorder of glycosphingolipid catabolism resulting from a deficiency of the enzyme alpha-galactosidase A, and leading to the progressive accumulation of one biomarker, globotriaosylceramide (Gb(3)), predominantly elevated in the urine of these patients.	globotriaosylceramide	215-236	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	238-243
17336267-7	2007	Thus, the binding of Gb(3)/CD77 by its cognate ligand transmits information within the lipid bilayer of model lymphoma cells to impact the behaviour of selective proteins, most notably CD20, via a mechanism influenced by the level of cholesterol within the membrane.	globotriaosylceramide	21-26	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	27-31
17336267-7	2007	Thus, the binding of Gb(3)/CD77 by its cognate ligand transmits information within the lipid bilayer of model lymphoma cells to impact the behaviour of selective proteins, most notably CD20, via a mechanism influenced by the level of cholesterol within the membrane.	globotriaosylceramide	21-26	keratin 20	Homo sapiens	185-189
17371887-1	2007	Fabry disease is an X-linked, hereditary, lysosomal storage disease caused by deficiency of the enzyme alpha-galactosidase A, which results in the accumulation of the neutral glycosphingolipid globotriaosylceramide (Gb3) in the walls of small blood vessels, nerves, dorsal root ganglia, renal glomerular and tubular epithelial cells, and cardiomyocytes.	globotriaosylceramide	193-214	galactosidase alpha	Homo sapiens	103-124
17371887-1	2007	Fabry disease is an X-linked, hereditary, lysosomal storage disease caused by deficiency of the enzyme alpha-galactosidase A, which results in the accumulation of the neutral glycosphingolipid globotriaosylceramide (Gb3) in the walls of small blood vessels, nerves, dorsal root ganglia, renal glomerular and tubular epithelial cells, and cardiomyocytes.	globotriaosylceramide	193-214	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	216-219
17287429-1	2007	Deficiency of lysosomal alpha-galactosidase A (alpha-Gal A) in Fabry disease results in cellular accumulation of globotriaosylceramide (Gl3), often leading to end-stage renal failure.	globotriaosylceramide	113-134	galactosidase alpha	Homo sapiens	24-45
17287429-1	2007	Deficiency of lysosomal alpha-galactosidase A (alpha-Gal A) in Fabry disease results in cellular accumulation of globotriaosylceramide (Gl3), often leading to end-stage renal failure.	globotriaosylceramide	113-134	galactosidase alpha	Homo sapiens	47-58
16991089-1	2006	Fabry disease is an X-linked recessive disorder in which affected persons lack alpha-galactosidase A (alpha -GalA), which leads to excess glycosphingolipids in tissues, mainly globotriaosylceramide (Gb3).	globotriaosylceramide	176-197	galactosidase alpha	Homo sapiens	79-100
16991089-1	2006	Fabry disease is an X-linked recessive disorder in which affected persons lack alpha-galactosidase A (alpha -GalA), which leads to excess glycosphingolipids in tissues, mainly globotriaosylceramide (Gb3).	globotriaosylceramide	176-197	galactosidase alpha	Homo sapiens	102-113
16991089-1	2006	Fabry disease is an X-linked recessive disorder in which affected persons lack alpha-galactosidase A (alpha -GalA), which leads to excess glycosphingolipids in tissues, mainly globotriaosylceramide (Gb3).	globotriaosylceramide	176-197	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	199-202
16898255-1	2006	The neurological manifestations of Fabry disease include both peripheral and central nervous system involvement caused by a deficiency of alpha-galactosidase A and accumulation of alpha-D-galactosyl moieties, particularly globotriosylceramide accumulation (Gb3).	globotriaosylceramide	222-242	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	257-260
17043001-2	2006	Lack of enzyme activity results in progressive accumulation of globotriaosylceramide (Gb3) leading to multiorgan dysfunction and early death.	globotriaosylceramide	63-84	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	86-89
17073606-1	2006	Fabry disease is caused by a deficiency of a-galactosidase A which leads to the progressive intra-lysosomal accumulation of ceramide trihexoside (CTH), also known as globotriaosylceramide (Gb3), in different cell types and body fluids.	globotriaosylceramide	166-187	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	189-192
16970764-1	2006	BACKGROUND: Fabry disease is an X-linked disorder due to a deficiency of alpha-galactosidase A and leads to the accumulation of globotriaosylceramide (Gb3) in various cells.	globotriaosylceramide	128-149	galactosidase alpha	Homo sapiens	73-94
16970764-1	2006	BACKGROUND: Fabry disease is an X-linked disorder due to a deficiency of alpha-galactosidase A and leads to the accumulation of globotriaosylceramide (Gb3) in various cells.	globotriaosylceramide	128-149	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	151-154
16439866-1	2006	OBJECTIVE: To determine the effect of a gp120 binding, non-cytotoxic soluble analogue of the glycosphingolipid (GSL), globotriaosyl ceramide (Gb3) on HIV infection in vitro.	globotriaosylceramide	118-140	inter-alpha-trypsin inhibitor heavy chain 4	Homo sapiens	40-45
16713681-5	2006	The peptide bound strongly to Stx-1 and Stx-2, though the binding was inhibited with Gb3Cer.	globotriaosylceramide	85-91	syntaxin 1A	Homo sapiens	30-35
16713681-5	2006	The peptide bound strongly to Stx-1 and Stx-2, though the binding was inhibited with Gb3Cer.	globotriaosylceramide	85-91	syntaxin 2	Homo sapiens	40-45
16595957-1	2006	A two-step binding assay for globotriaosylceramide (Gb3) content was developed by histidine-tagging strategy, which is a well-established method for the purification of recombinant proteins.	globotriaosylceramide	29-50	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	52-55
16609685-1	2006	Enzyme replacement therapy (ERT) with recombinant human alpha-galactosidase A (r-halphaGalA) enhances microvascular globotriaosylceramide clearance and improves clinical symptoms in patients with Fabry disease.	globotriaosylceramide	116-137	galactosidase alpha	Homo sapiens	56-77
16495579-1	2006	We previously developed linear polymers bearing clustered trisaccharides of globotriaosylceramide (Gb3) as orally applicable Shiga toxin (Stx) neutralizers.	globotriaosylceramide	76-97	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	99-102
21290687-1	2006	Globotriaosylceramide (Gb3) deposits are found in nearly all cardiac structures, including the myocardium, endocardium, endothelium and conduction cells, and in the autonomic nervous system.	globotriaosylceramide	0-21	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	23-26
16601877-1	2006	Fabry disease (McKusick 301500) is an X-linked lysosomal storage disorder secondary to deficient alpha-galactosidase A activity which leads to the widespread accumulation of globotriaosylceramide (Gb(3)) and related glycosphingolipids, especially in vascular smooth-muscle and endothelial cells.	globotriaosylceramide	174-195	galactosidase alpha	Homo sapiens	97-118
21290697-2	2006	All classic hemizygotes and over 90% of heterozygotes have an elevated level of urinary globotriaosylceramide (Gb3 ).	globotriaosylceramide	88-109	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	111-114
16403380-4	2005	Fabry disease is an x-linked recessive disorder in which deficiency of the lysosomal enzyme alpha-galactosidase A leads to progressive accumulation of globotriaosylceramide in vital organs.	globotriaosylceramide	151-172	galactosidase alpha	Homo sapiens	92-113
16432775-2	2006	In consequence, globotriaosylceramide (Gb3) accumulates in nearly all tissues and body fluids.	globotriaosylceramide	16-37	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	39-42
16802573-3	2006	With increasing age globotriaosylceramide (Gb3) progressively accumulates in different cells, tissues and organs throughout the body.	globotriaosylceramide	20-41	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	43-46
16287034-2	2006	Globotriaosylceramides (Gb(3)) are biological compounds implicated in Fabry disease, a lysosomal storage disease due to the deficient activity of alpha-D-galactosidase A, which results in an accumulation of Gb(3) in many organs.	globotriaosylceramide	0-22	galactosidase alpha	Homo sapiens	146-169
16287034-2	2006	Globotriaosylceramides (Gb(3)) are biological compounds implicated in Fabry disease, a lysosomal storage disease due to the deficient activity of alpha-D-galactosidase A, which results in an accumulation of Gb(3) in many organs.	globotriaosylceramide	24-29	galactosidase alpha	Homo sapiens	146-169
16287034-2	2006	Globotriaosylceramides (Gb(3)) are biological compounds implicated in Fabry disease, a lysosomal storage disease due to the deficient activity of alpha-D-galactosidase A, which results in an accumulation of Gb(3) in many organs.	globotriaosylceramide	207-212	galactosidase alpha	Homo sapiens	146-169
17236300-1	2006	Anderson Fabry disease (alpha galactosidase A deficiency) is an X-linked recessive lysosomal storage disorder; alpha galactosidase A deficiency results in accumulation of neutral glycosphingolipids, especially globotriaosylceramide (Gb3), in various cell types promoting development of disease with renal, cardiovascular, and cerebrovascular involvement.	globotriaosylceramide	210-231	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	233-236
16365318-6	2005	Transfection of Gb(3) synthase, resulting in Gb(3) expression in noncancerous polarized epithelial cells lacking endogenous Gb(3), induced cell invasiveness.	globotriaosylceramide	45-50	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	16-30
16148726-1	2005	Fabry disease is a rare X-linked lysosomal storage disorder caused by deficient activity of alpha-galactosidase A (alpha-Gal A) resulting in the storage of glycosphingolipids, especially globotriaosylceramide (Gb3), in cells throughout the body, causing life-threatening renal, cardiac, and cerebrovascular complications in hemizygous males and some heterozygous females.	globotriaosylceramide	187-208	galactosidase alpha	Homo sapiens	92-113
16148726-1	2005	Fabry disease is a rare X-linked lysosomal storage disorder caused by deficient activity of alpha-galactosidase A (alpha-Gal A) resulting in the storage of glycosphingolipids, especially globotriaosylceramide (Gb3), in cells throughout the body, causing life-threatening renal, cardiac, and cerebrovascular complications in hemizygous males and some heterozygous females.	globotriaosylceramide	187-208	galactosidase alpha	Homo sapiens	115-126
16148726-1	2005	Fabry disease is a rare X-linked lysosomal storage disorder caused by deficient activity of alpha-galactosidase A (alpha-Gal A) resulting in the storage of glycosphingolipids, especially globotriaosylceramide (Gb3), in cells throughout the body, causing life-threatening renal, cardiac, and cerebrovascular complications in hemizygous males and some heterozygous females.	globotriaosylceramide	187-208	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	210-213
15895708-1	2005	UNLABELLED: Fabry disease is a rare, X-linked lysosomal storage disease caused by an inborn deficiency of alpha-galactosidase A, which results in progressive accumulation of globotriaosylceramide in a range of cells and tissues.	globotriaosylceramide	174-195	galactosidase alpha	Homo sapiens	106-127
16210890-1	2005	BACKGROUND: Fabry disease is a rare X-linked disorder that results from a deficiency in a lysosomal enzyme known as alpha-galactosidase A, with accumulation of globotriaosylceramide (Gl3).	globotriaosylceramide	160-181	galactosidase alpha	Homo sapiens	116-137
15959771-1	2005	Fabry disease (FD) is an X-linked inborn error of glycosphingolipid (GSL) metabolism, caused by a deficiency of the lysosomal alpha-galactosidase A, which results in high levels in lysosomes and biological fluids of globotriaosylceramide (Gb3) and digalactosylceramide (Ga2), also known as galabiosylceramide.	globotriaosylceramide	216-237	galactosidase alpha	Homo sapiens	126-147
16165112-3	2005	The populations express high, medium, and low levels of globotriaosylceramide as determined by the binding of the Stx1B reagent.	globotriaosylceramide	56-77	syntaxin 1B	Homo sapiens	114-119
15744065-1	2005	Rhamnose-binding lectin from catfish (Silurus asotus) eggs (SAL) has the ability to induce externalization of phosphatidylserine (PS), followed by cell shrinkage in globotriaosylceramide (Gb3)-expressing Burkitt"s lymphoma Raji cells.	globotriaosylceramide	165-186	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	188-191
15895713-1	2005	AIM: The aim of this study was to determine whether globotriaosylceramide (Gb3) is a useful biomarker in Fabry disease.	globotriaosylceramide	52-73	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	75-78
15880667-1	2005	Globotriaosylceramide is a neutral glycolipid containing the trihexoside Gal(alpha1-4)Gal(ss1-4)Glc(ss1-1") covalently bound to N-acylsphingosine.	globotriaosylceramide	0-21	adrenoceptor alpha 1D	Homo sapiens	77-85
16151903-2	2005	It results from a deficiency of the lysosomal alpha-galactosidase A and leads to progressive accumulation of globotriaosylceramide in the endothelium and tissue cells of various organs.	globotriaosylceramide	109-130	galactosidase alpha	Homo sapiens	46-67
15320778-2	2004	The disease occurs due to deficient activity of alpha-galactosidase A, leading to progressive accumulation of globotriaosylceramide in multiple organs and tissues.	globotriaosylceramide	110-131	galactosidase alpha	Homo sapiens	48-69
15702403-1	2005	Anderson-Fabry disease (referred to as Fabry disease) is an X-linked disorder characterized by a deficiency of the lysosomal enzyme alpha-galactosidase A and the subsequent accumulation in various tissues of globotriaosylceramide (Gb(3)), the main substrate of the defective enzyme.	globotriaosylceramide	231-236	galactosidase alpha	Homo sapiens	132-153
15702403-9	2005	In one patient, after several months of ERT, there was a transient increase in Gb(3) concentrations to baseline (pre-ERT) levels, associated with the presence of antibodies to the recombinant alpha-galactosidase A.	globotriaosylceramide	79-84	galactosidase alpha	Homo sapiens	192-213
15702404-2	2005	This deficiency leads to the progressive accumulation, in lysosomes of visceral tissues and in body fluids of hemizygotes, of the glycosphingolipids globotriaosylceramide (CTH, Gb(3) or GL-3) and galabiosylceramide (CDH) and to a lesser extent the blood group AB and B related glycolipids.	globotriaosylceramide	149-170	V-set and immunoglobulin domain containing 2	Homo sapiens	172-175
14989466-1	2003	AIM: To evaluate the sequelae of the lysosomal storage of globotriaosylceramide (Gb3) in a series of patients with Fabry disease.	globotriaosylceramide	58-79	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	81-84
15039308-5	2004	The majority of Gb(3)/CD77(+) cells were activated CD3(+) CD6(+) CD8 alpha(+) T cells, whereas only some CD4(+) T cells and B cells expressed Gb(3)/CD77.	globotriaosylceramide	16-21	CD8a molecule	Bos taurus	65-74
15102080-2	2004	These disturbances are caused by cellular globotriaosylceramide accumulation in the skin due to deficient lysosomal alpha-galactosidase A activity.	globotriaosylceramide	42-63	galactosidase alpha	Homo sapiens	116-137
14716680-1	2004	BACKGROUND: Fabry disease is a recessive, X-linked disorder caused by a deficiency of the lysosomal enzyme alpha-galactosidase A, leading to an accumulation of the glycosphingolipid globotriaosylceramide (GL-3) in most tissues of the body.	globotriaosylceramide	182-203	galactosidase, alpha	Mus musculus	107-128
15458455-1	2004	BACKGROUND: Fabry disease is an X-linked inherited disorder that is caused by excessive lysosomal globotriaosylceramide (CTH) storage due to a deficiency in alpha-galactosidase A (alpha-Gal A).	globotriaosylceramide	98-119	galactosidase alpha	Homo sapiens	157-178
15458455-1	2004	BACKGROUND: Fabry disease is an X-linked inherited disorder that is caused by excessive lysosomal globotriaosylceramide (CTH) storage due to a deficiency in alpha-galactosidase A (alpha-Gal A).	globotriaosylceramide	98-119	galactosidase alpha	Homo sapiens	180-191
15458455-1	2004	BACKGROUND: Fabry disease is an X-linked inherited disorder that is caused by excessive lysosomal globotriaosylceramide (CTH) storage due to a deficiency in alpha-galactosidase A (alpha-Gal A).	globotriaosylceramide	121-124	galactosidase alpha	Homo sapiens	157-178
15458455-1	2004	BACKGROUND: Fabry disease is an X-linked inherited disorder that is caused by excessive lysosomal globotriaosylceramide (CTH) storage due to a deficiency in alpha-galactosidase A (alpha-Gal A).	globotriaosylceramide	121-124	galactosidase alpha	Homo sapiens	180-191
15288673-5	2004	The main substance accumulated is globotriaosylceramide (Gb3).	globotriaosylceramide	34-55	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	57-60
15135109-8	2004	Finally, this method was applied to detect the excess of one of the neutral sphingolipids, namely globotriaosylceramide (Gb3) in the urine of patients affected with Fabry disease.	globotriaosylceramide	98-119	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	121-124
15365954-2	2004	The deficiency leads to progressive glycosphingolipid globotriaosylceramide (Gb3) accumulation in fluids and tissues, including vascular endothelium, connective tissue, kidney, heart, brain and peripheral nerves.	globotriaosylceramide	54-75	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	77-80
14973368-1	2004	Purified renal globotriaosyl ceramide (Gb3)/cholesterol mixtures sonicated heated in a Triton-containing buffer placed below a discontinuous sucrose gradient form glycosphingolipid (GSL)-containing dense lipid structures at the 30/5% sucrose interface after centrifugation.	globotriaosylceramide	15-37	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	39-42
12624185-1	2003	Fabry disease is an X-linked recessive inborn metabolic disorder characterized by systemic and vascular accumulation of globotriaosylceramide (Gb(3)) caused by a deficiency of the lysosomal enzyme alpha-galactosidase A (alpha-gal A).	globotriaosylceramide	120-141	galactosidase, alpha	Mus musculus	197-218
12624185-1	2003	Fabry disease is an X-linked recessive inborn metabolic disorder characterized by systemic and vascular accumulation of globotriaosylceramide (Gb(3)) caused by a deficiency of the lysosomal enzyme alpha-galactosidase A (alpha-gal A).	globotriaosylceramide	120-141	galactosidase, alpha	Mus musculus	220-231
12624185-1	2003	Fabry disease is an X-linked recessive inborn metabolic disorder characterized by systemic and vascular accumulation of globotriaosylceramide (Gb(3)) caused by a deficiency of the lysosomal enzyme alpha-galactosidase A (alpha-gal A).	globotriaosylceramide	143-148	galactosidase, alpha	Mus musculus	197-218
12624185-1	2003	Fabry disease is an X-linked recessive inborn metabolic disorder characterized by systemic and vascular accumulation of globotriaosylceramide (Gb(3)) caused by a deficiency of the lysosomal enzyme alpha-galactosidase A (alpha-gal A).	globotriaosylceramide	143-148	galactosidase, alpha	Mus musculus	220-231
12580945-2	2003	The potent cytotoxic activity of VTs is important in pathogenicity, resulting in the death of cells expressing receptor Gb3 (globotriaosylceramide).	globotriaosylceramide	125-146	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	120-123
12645583-7	2003	Analysis of their structures with glycosphingolipid-specific antibodies and negative secondary ion mass spectrometry identified them as globotriaosylceramide (Gb3) and globotetraosylceramide (Gb4).	globotriaosylceramide	136-157	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	159-162
12364551-0	2002	A novel soluble analog of the HIV-1 fusion cofactor, globotriaosylceramide (Gb(3)), eliminates the cholesterol requirement for high affinity gp120/Gb(3) interaction.	globotriaosylceramide	53-74	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	76-81
12538729-5	2003	In this model, Gla-/0 mice displayed a progressive age-dependent shortening of the time to occlusive thrombosis after vascular injury that correlated with progressive accumulation of globotriasylceramide (Gb3) in the arterial wall.	globotriaosylceramide	205-208	galactosidase, alpha	Mus musculus	15-18
12540565-6	2003	Closely following the change in ceramide level was an increase in the expression of globotriaosylceramide (Gb3), the receptor for Stx2.	globotriaosylceramide	84-105	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	107-110
12540565-6	2003	Closely following the change in ceramide level was an increase in the expression of globotriaosylceramide (Gb3), the receptor for Stx2.	globotriaosylceramide	84-105	syntaxin 2	Homo sapiens	130-134
12413469-0	2002	Self-assembled monolayers of globotriaosylceramide (Gb3) mimics: surface-specific affinity with shiga toxins.	globotriaosylceramide	29-50	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	52-55
12232838-3	2002	However, exposure of human brain endothelial cells (HBECs) to tumor necrosis factor (TNF) and/or interleukin (IL)-1 markedly up-regulates Stx receptor (globotriaosylceramide; Gb3) expression and cytotoxicity.	globotriaosylceramide	152-173	tumor necrosis factor	Homo sapiens	62-83
12232838-3	2002	However, exposure of human brain endothelial cells (HBECs) to tumor necrosis factor (TNF) and/or interleukin (IL)-1 markedly up-regulates Stx receptor (globotriaosylceramide; Gb3) expression and cytotoxicity.	globotriaosylceramide	152-173	tumor necrosis factor	Homo sapiens	85-88
12232838-3	2002	However, exposure of human brain endothelial cells (HBECs) to tumor necrosis factor (TNF) and/or interleukin (IL)-1 markedly up-regulates Stx receptor (globotriaosylceramide; Gb3) expression and cytotoxicity.	globotriaosylceramide	152-173	interleukin 1 alpha	Homo sapiens	97-115
12364551-0	2002	A novel soluble analog of the HIV-1 fusion cofactor, globotriaosylceramide (Gb(3)), eliminates the cholesterol requirement for high affinity gp120/Gb(3) interaction.	globotriaosylceramide	53-74	inter-alpha-trypsin inhibitor heavy chain 4	Homo sapiens	141-146
12364551-0	2002	A novel soluble analog of the HIV-1 fusion cofactor, globotriaosylceramide (Gb(3)), eliminates the cholesterol requirement for high affinity gp120/Gb(3) interaction.	globotriaosylceramide	53-74	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	147-152
12395219-2	2002	The deficiency of alpha-galactosidase A leads to a progressive accumulation of globotriaosylceramide (Gb(3)), the major glycosphingolipid substrate of the enzyme, within vulnerable cells, tissues, and organs, including the cardiovascular system.	globotriaosylceramide	79-100	galactosidase alpha	Homo sapiens	18-39
12395219-2	2002	The deficiency of alpha-galactosidase A leads to a progressive accumulation of globotriaosylceramide (Gb(3)), the major glycosphingolipid substrate of the enzyme, within vulnerable cells, tissues, and organs, including the cardiovascular system.	globotriaosylceramide	102-108	galactosidase alpha	Homo sapiens	18-39
11838967-2	2001	Globotriaosyl ceramide (CD77), a marker for germinal center B-cells, is present on Daudi cells but is deficient in the Daudi-derived mutant VT500 cell line.	globotriaosylceramide	0-22	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	24-28
12082546-2	2002	Verotoxin 1 (VT1) is an Escherichia coli toxin, which has recently been shown to have anti-neoplastic action by targeting the globotriosylceramide (Gb(3)) glycolipid on tumor cells and tumor neovasculature.	globotriaosylceramide	126-146	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	148-153
12053080-1	2002	Anderson-Fabry disease (AFd) is a rare X-linked disorder characterized by deficiency of alpha-galactosidase A that leads to systemic accumulation of neutral glycosphingolipids, predominantly globotriaosylceramide (Gb3), in body fluids and visceral tissues, including the kidney.	globotriaosylceramide	191-212	galactosidase alpha	Homo sapiens	88-109
12203240-1	2002	A method for measuring globotriaosylceramide (Gb3, or GL3) levels in plasma and urine of humans affected by Anderson-Fabry disease has been developed.	globotriaosylceramide	23-44	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	46-49
11908825-1	2001	We determined the binding of verotoxin-1 (VT1) and verotoxin-2 (VT2) against globotriaosylceramide (Gb3) by a monoclonal antibody (mAb)-based enzyme-linked immunosorbent assay (ELISA).	globotriaosylceramide	77-98	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	100-103
12191968-1	2002	Cellular injury in post-diarrheal hemolytic-uremic syndrome (D+HUS) is related to shigatoxin (Stx) binding to globotriaosylceramide (Gb3).	globotriaosylceramide	110-131	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	133-136
12374217-5	2002	Nonlinear regression analysis of direct binding assay to these Gb3 isoforms confirmed the increased affinity of both toxins for the C22 hydroxylated Gb3.	globotriaosylceramide	63-66	Sp7 transcription factor 7	Mus musculus	132-135
12027559-3	2002	Fabry disease is caused by a deficiency of the lysosomal hydrolase alpha-galactosidase A, resulting in the abnormal deposition of the glycosphingolipid globotriaosylceramide (GL-3) in vascular lysosomes.	globotriaosylceramide	152-173	galactosidase, alpha	Mus musculus	67-88
11890871-1	2002	Anderson-Fabry disease (AFD) is a lysosomal storage disorder (LSD) due to alpha-galactosidase A (alpha-Gal A) deficiency and the resultant accumulation of incompletely metabolised glycosphingolipids (GSLs), primarily globotriosylceramide (Gb(3)), within various tissues.	globotriaosylceramide	217-237	galactosidase alpha	Homo sapiens	97-108
11890871-1	2002	Anderson-Fabry disease (AFD) is a lysosomal storage disorder (LSD) due to alpha-galactosidase A (alpha-Gal A) deficiency and the resultant accumulation of incompletely metabolised glycosphingolipids (GSLs), primarily globotriosylceramide (Gb(3)), within various tissues.	globotriaosylceramide	239-245	galactosidase alpha	Homo sapiens	97-108
11726142-3	2001	Attachment of the B subunit of VTs to its receptor, globotriaosylceramide (Gb3), at gut epithelium is the primary step and, consequently, the A subunit of VTs inhibits protein synthesis in the target cell.	globotriaosylceramide	52-73	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	75-78
11453701-8	2001	One possibility for this is that the more extensive binding of the Stx to the kidney tissue of the paediatric patient might be due to the higher synthesis and expression of Stx receptors, i.e. Gb(3), in infants and less so in the aged individuals.	globotriaosylceramide	193-198	ST8 alpha-N-acetyl-neuraminide alpha-2,8-sialyltransferase 2	Homo sapiens	67-70
11604159-0	2001	Globotriaosylceramide (Gb(3)/CD77) is synthesized and surface expressed by bovine lymphocytes upon activation in vitro.	globotriaosylceramide	0-21	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	29-33
11604159-12	2001	However, stimulation induced an increase of CMH and Gb(3) by a factor of 2.5 and 10, respectively, implicating that bovine lymphocytes regulate surface expression of Gb(3)/CD77 predominantly by quantitative changes in the Gb(3) metabolism.	globotriaosylceramide	166-171	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	172-176
11604159-12	2001	However, stimulation induced an increase of CMH and Gb(3) by a factor of 2.5 and 10, respectively, implicating that bovine lymphocytes regulate surface expression of Gb(3)/CD77 predominantly by quantitative changes in the Gb(3) metabolism.	globotriaosylceramide	166-171	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	172-176
11604159-13	2001	This report presents Gb(3)/CD77 as the first GSL identified on bovine immune cells and highly recommends this activation dependent antigen as a useful tool to investigate lymphocyte activation within the bovine immune system.	globotriaosylceramide	21-26	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	27-31
11571244-1	2001	BACKGROUND: Fabry disease is an X-linked lysosomal deficiency of alpha-galactosidase A that results in cellular accumulation of galacto-conjugates such as globotriosylceramide, particularly in blood vessels.	globotriaosylceramide	155-175	galactosidase alpha	Homo sapiens	65-86
11453701-8	2001	One possibility for this is that the more extensive binding of the Stx to the kidney tissue of the paediatric patient might be due to the higher synthesis and expression of Stx receptors, i.e. Gb(3), in infants and less so in the aged individuals.	globotriaosylceramide	193-198	ST8 alpha-N-acetyl-neuraminide alpha-2,8-sialyltransferase 2	Homo sapiens	173-176
11439963-8	2001	Patients in the recombinant alpha-galactosidase A group also had decreased microvascular endothelial deposits of globotriaosylceramide in the skin (P<0.001) and heart (P<0.001).	globotriaosylceramide	113-134	galactosidase alpha	Homo sapiens	28-49
11439963-10	2001	After six months of open-label therapy, all patients in the former placebo group and 98 percent of patients in the former recombinant alpha-galactosidase A group who had biopsies had clearance of microvascular endothelial deposits of globotriaosylceramide.	globotriaosylceramide	234-255	galactosidase alpha	Homo sapiens	134-155
11439963-13	2001	CONCLUSIONS: Recombinant alpha-galactosidase A replacement therapy cleared microvascular endothelial deposits of globotriaosylceramide from the kidneys, heart, and skin in patients with Fabry"s disease, reversing the pathogenesis of the chief clinical manifestations of this disease.	globotriaosylceramide	113-134	galactosidase alpha	Homo sapiens	25-46
11369233-3	2001	SLT-1 binds to the glycolipid globotriaosylceramide [3, 4], which acts as a shuttle, allowing the toxin to be imported and routed near ribosomes.	globotriaosylceramide	30-51	unconventional SNARE in the ER 1	Homo sapiens	0-5
11437603-1	2001	A new single-step purification method for Shiga toxin (Stx) was developed using receptor-mediated affinity chromatography, in which Gb3Cer (globotriaosylceramide) was conjugated to octyl Sepharose CL-4B as a carrier.	globotriaosylceramide	140-161	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	132-135
11418306-1	2001	The ribosome-inactivating protein, Shiga-like toxin-1 (SLT-1, SLT-I, Verotoxin 1, VT1) targets cells that express the glycolipid globotriaosylceramide (CD77) on their surface.	globotriaosylceramide	129-150	unconventional SNARE in the ER 1	Homo sapiens	35-53
11418306-1	2001	The ribosome-inactivating protein, Shiga-like toxin-1 (SLT-1, SLT-I, Verotoxin 1, VT1) targets cells that express the glycolipid globotriaosylceramide (CD77) on their surface.	globotriaosylceramide	129-150	unconventional SNARE in the ER 1	Homo sapiens	55-60
11418306-1	2001	The ribosome-inactivating protein, Shiga-like toxin-1 (SLT-1, SLT-I, Verotoxin 1, VT1) targets cells that express the glycolipid globotriaosylceramide (CD77) on their surface.	globotriaosylceramide	129-150	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	152-156
11264150-6	2001	Exogenously added GlcCer and the homologous Glc-containing globotriaosylceramide (Gb3Cer), but not galactosylceramide, dose-dependently prolonged clotting times of normal plasma in the presence, but not absence, of APC:protein S, which suggests that GlcCer or Gb3Cer can enhance protein C pathway anticoagulant activity.	globotriaosylceramide	59-80	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	82-85
11158156-1	2001	The relationship between differential susceptibility to hemolytic-uremic syndrome (HUS) and levels of globotriaosylceramide (Gb3) in serum was studied in patients infected with verotoxin-producing Escherichia coli (VTEC).	globotriaosylceramide	102-123	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	125-128
11249924-0	2001	Quantified increases of cholesterol, total lipid and globotriaosylceramide in filipin-positive Niemann-Pick type C fibroblasts.	globotriaosylceramide	53-74	protein interacting with PRKCA 1	Homo sapiens	103-107
11249924-7	2001	Another increase found in the NPC cells was that of a minor lipid fraction, globotriaosylceramide (Gb3, known as a cell signalling glycolipid).	globotriaosylceramide	76-97	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	99-102
11201791-6	2000	Using this approach, we identified globotriaosylceramide (Gb3) and ganglioside GM3 as the main lymphocyte GSL recognized by gp120.	globotriaosylceramide	35-56	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	58-61
11758681-2	2001	This leads to systemic accumulation of the glycosphingolipid globotriaosylceramide (Gb3) in all body tissues and organs, including the kidney.	globotriaosylceramide	61-82	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	84-87
11063874-0	2000	Globotriaosyl ceramide (CD77/Gb3) in the glycolipid-enriched membrane domain participates in B-cell receptor-mediated apoptosis by regulating lyn kinase activity in human B cells.	globotriaosylceramide	0-22	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	24-28
11063874-0	2000	Globotriaosyl ceramide (CD77/Gb3) in the glycolipid-enriched membrane domain participates in B-cell receptor-mediated apoptosis by regulating lyn kinase activity in human B cells.	globotriaosylceramide	0-22	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	29-32
11063874-0	2000	Globotriaosyl ceramide (CD77/Gb3) in the glycolipid-enriched membrane domain participates in B-cell receptor-mediated apoptosis by regulating lyn kinase activity in human B cells.	globotriaosylceramide	0-22	LYN proto-oncogene, Src family tyrosine kinase	Homo sapiens	142-145
10801274-1	2000	Human intestinal cells lack globotriaosylceramide (Gb(3)), the receptor for Shiga toxin-1 (Stx1) and Shiga toxin-2 (Stx2).	globotriaosylceramide	51-56	syntaxin 1A	Homo sapiens	91-95
10801274-4	2000	Stx1 (12.5 ng/ml) induced apoptosis in both HEp-2 (21.9 +/- 7.9% vs. 0.8 +/- 0.3%, P = 0.01) and Caco-2 (10.1 +/- 1.2% vs. 3.1 +/- 0.4%, P = 0.006) cells but not in Gb(3)-deficient T84 cells.	globotriaosylceramide	165-170	syntaxin 1A	Homo sapiens	0-4
10840053-2	2000	A deficiency in the lysosomal hydrolase alpha-galactosidase A (alpha-gal A; EC ) leads to impaired catabolism of alpha-galactosyl-terminal lipids such as globotriaosylceramide (Gb3).	globotriaosylceramide	154-175	galactosidase, alpha	Mus musculus	40-61
10840053-2	2000	A deficiency in the lysosomal hydrolase alpha-galactosidase A (alpha-gal A; EC ) leads to impaired catabolism of alpha-galactosyl-terminal lipids such as globotriaosylceramide (Gb3).	globotriaosylceramide	154-175	galactosidase, alpha	Mus musculus	63-74
10840053-2	2000	A deficiency in the lysosomal hydrolase alpha-galactosidase A (alpha-gal A; EC ) leads to impaired catabolism of alpha-galactosyl-terminal lipids such as globotriaosylceramide (Gb3).	globotriaosylceramide	177-180	galactosidase, alpha	Mus musculus	40-61
10840053-2	2000	A deficiency in the lysosomal hydrolase alpha-galactosidase A (alpha-gal A; EC ) leads to impaired catabolism of alpha-galactosyl-terminal lipids such as globotriaosylceramide (Gb3).	globotriaosylceramide	177-180	galactosidase, alpha	Mus musculus	63-74
10841515-4	2000	A concentration-dependent decrement in renal and hepatic globotriaosylceramide levels was observed in alpha-Gal A(-) males treated for 4 weeks with D-t-EtDO-P4.	globotriaosylceramide	57-78	galactosidase, alpha	Mus musculus	102-113
10841515-5	2000	When 8-week-old alpha-Gal A(-) males were treated for 8 weeks with 10 mg/kg twice daily, renal globotriaosylceramide fell to below starting levels, consistent with an alpha-galactosidase A-independent salvage pathway for globotriaosylceramide degradation.	globotriaosylceramide	95-116	galactosidase, alpha	Mus musculus	16-27
11201791-6	2000	Using this approach, we identified globotriaosylceramide (Gb3) and ganglioside GM3 as the main lymphocyte GSL recognized by gp120.	globotriaosylceramide	35-56	inter-alpha-trypsin inhibitor heavy chain 4	Homo sapiens	124-129
10652021-9	2000	CONCLUSIONS: These findings suggest that selective glucosylceramide synthase inhibitors are highly effective in the depletion of globotriaosylceramide from Fabry cell lines.	globotriaosylceramide	129-150	UDP-glucose ceramide glucosyltransferase	Homo sapiens	51-76
10575015-0	1999	Activation of Src family kinase yes induced by Shiga toxin binding to globotriaosyl ceramide (Gb3/CD77) in low density, detergent-insoluble microdomains.	globotriaosylceramide	70-92	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	94-97
10567921-8	2000	Unlike VT1 cytotoxicity, IFN-alpha-induced Stat1 nuclear translocation was not inhibited when RME was prevented, suggesting that the accessory function of Gb(3) occurs at the plasma membrane.	globotriaosylceramide	155-160	signal transducer and activator of transcription 1	Homo sapiens	43-48
10567921-10	2000	In both systems, long chain fatty acid-containing Gb(3) isoforms, which are less effective to mediate VT1 cytotoxicity, were found to correlate with higher IFN-alpha-mediated antiviral activity.	globotriaosylceramide	50-55	interferon alpha 2	Homo sapiens	156-165
10567921-11	2000	Inhibition of Gb(3) synthesis in toto prevented IFN-alpha antiviral activity in all cells.	globotriaosylceramide	14-19	interferon alpha 2	Homo sapiens	48-57
10567921-12	2000	We propose that the long chain Gb(3) fatty isoforms preferentially remain in the plasma membrane, and by associating with IFNAR1, mediate IFN-alpha antiviral signaling, whereas short chain Gb(3) fatty acid isoforms are preferentially internalized to mediate VT1 cytotoxicity and IFNAR1-dependent IFN-alpha growth inhibition.	globotriaosylceramide	31-36	interferon alpha 2	Homo sapiens	138-147
10658586-3	1999	Neoglycopeptides generated were tested for the inhibition of verotoxin binding to globotriosylceramide (Gb3) using ELISA.	globotriaosylceramide	82-102	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	104-107
10692420-0	2000	Retroviral transfection of Madin-Darby canine kidney cells with human MDR1 results in a major increase in globotriaosylceramide and 10(5)- to 10(6)-fold increased cell sensitivity to verocytotoxin.	globotriaosylceramide	106-127	ATP binding cassette subfamily B member 1	Homo sapiens	70-74
10692420-4	2000	P-gp (but not MRP) inhibitors, e.g. ketoconazole or cyclosporin A (CsA) prevented the increased Gb(3) and VT sensitivity, concomitant with increased vinblastine sensitivity.	globotriaosylceramide	96-101	PGP	Canis lupus familiaris	0-4
10618424-9	2000	After the single dose of alpha-gal A, nine of the 10 patients had significantly reduced Gb(3) levels both in the liver and shed renal tubular epithelial cells in the urine sediment.	globotriaosylceramide	88-93	galactosidase alpha	Homo sapiens	25-36
10567921-0	2000	Functional significance of globotriaosyl ceramide in interferon-alpha(2)/type 1 interferon receptor-mediated antiviral activity.	globotriaosylceramide	27-49	interferon alpha 2	Homo sapiens	53-99
10567921-3	2000	Gb(3)-deficient variant cells selected for VT resistance are cross-resistant to interferon-alpha (IFN-alpha)-mediated antiproliferative activity.	globotriaosylceramide	0-5	interferon alpha 2	Homo sapiens	98-107
10567921-5	2000	The crucial role of Gb(3) for the signal transduction of IFN-alpha-mediated antiviral activity is now reported.	globotriaosylceramide	20-25	interferon alpha 2	Homo sapiens	57-66
10567921-6	2000	IFN-alpha-mediated antiviral activity, nuclear translocation of activated Stat1, and increased expression of PKR were defective in Gb(3)-deficient vero mutant cells, although the surface expression of IFNAR1 was unaltered.	globotriaosylceramide	131-136	interferon alpha-2	Chlorocebus sabaeus	0-9
10567921-6	2000	IFN-alpha-mediated antiviral activity, nuclear translocation of activated Stat1, and increased expression of PKR were defective in Gb(3)-deficient vero mutant cells, although the surface expression of IFNAR1 was unaltered.	globotriaosylceramide	131-136	eukaryotic translation initiation factor 2 alpha kinase 2	Homo sapiens	109-112
10567921-8	2000	Unlike VT1 cytotoxicity, IFN-alpha-induced Stat1 nuclear translocation was not inhibited when RME was prevented, suggesting that the accessory function of Gb(3) occurs at the plasma membrane.	globotriaosylceramide	155-160	interferon alpha 2	Homo sapiens	25-34
11133022-0	1999	Globotriaosyl ceramide modulates interferon-alpha-induced growth inhibition and CD19 expression in Burkitt"s lymphoma cells.	globotriaosylceramide	0-22	CD19 molecule	Homo sapiens	80-84
11133022-1	1999	Previous studies have indicated that globotriaosyl ceramide (Gb3 or CD77) plays a role in alpha-interferon signal transduction and CD19-mediated homotypic adhesion in B cell lines derived from Burkitt"s lymphoma.	globotriaosylceramide	37-59	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	61-64
11133022-1	1999	Previous studies have indicated that globotriaosyl ceramide (Gb3 or CD77) plays a role in alpha-interferon signal transduction and CD19-mediated homotypic adhesion in B cell lines derived from Burkitt"s lymphoma.	globotriaosylceramide	37-59	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	68-72
11133022-1	1999	Previous studies have indicated that globotriaosyl ceramide (Gb3 or CD77) plays a role in alpha-interferon signal transduction and CD19-mediated homotypic adhesion in B cell lines derived from Burkitt"s lymphoma.	globotriaosylceramide	37-59	CD19 molecule	Homo sapiens	131-135
10575015-0	1999	Activation of Src family kinase yes induced by Shiga toxin binding to globotriaosyl ceramide (Gb3/CD77) in low density, detergent-insoluble microdomains.	globotriaosylceramide	70-92	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	98-102
10575015-1	1999	Shiga toxin (Stx) is an enterotoxin produced by Shigella dysenteriae serotype 1 and enterohemorrhagic Escherichia coli, which binds specifically to globotriaosylceramide, Gb3, on the cell surface and causes cell death.	globotriaosylceramide	148-169	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	171-174
10515895-1	1999	The ribosome-inactivating protein, Shiga-like toxin-1 (SLT-1), targets cells that express the glycolipid globotriaosylceramide (CD77) on their surface.	globotriaosylceramide	105-126	unconventional SNARE in the ER 1	Homo sapiens	35-53
10515895-1	1999	The ribosome-inactivating protein, Shiga-like toxin-1 (SLT-1), targets cells that express the glycolipid globotriaosylceramide (CD77) on their surface.	globotriaosylceramide	105-126	unconventional SNARE in the ER 1	Homo sapiens	55-60
10515895-1	1999	The ribosome-inactivating protein, Shiga-like toxin-1 (SLT-1), targets cells that express the glycolipid globotriaosylceramide (CD77) on their surface.	globotriaosylceramide	105-126	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	128-132
10233996-3	1999	We identified globotriaosylceramide (Gb3) and ganglioside GM3 as the main glycosphingolipids recognized by gp120.	globotriaosylceramide	14-35	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	37-40
10589997-0	1999	Role of glycosphingolipids in HIV-1 entry: requirement of globotriosylceramide (Gb3) in CD4/CXCR4-dependent fusion.	globotriaosylceramide	58-78	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	80-83
10589997-0	1999	Role of glycosphingolipids in HIV-1 entry: requirement of globotriosylceramide (Gb3) in CD4/CXCR4-dependent fusion.	globotriaosylceramide	58-78	CD4 molecule	Homo sapiens	88-91
10589997-0	1999	Role of glycosphingolipids in HIV-1 entry: requirement of globotriosylceramide (Gb3) in CD4/CXCR4-dependent fusion.	globotriaosylceramide	58-78	C-X-C motif chemokine receptor 4	Homo sapiens	92-97
10540335-0	1999	The extrafollicular-to-follicular transition of human B lymphocytes: induction of functional globotriaosylceramide (CD77) on high threshold occupancy of CD40.	globotriaosylceramide	93-114	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	116-120
10540335-0	1999	The extrafollicular-to-follicular transition of human B lymphocytes: induction of functional globotriaosylceramide (CD77) on high threshold occupancy of CD40.	globotriaosylceramide	93-114	CD40 molecule	Homo sapiens	153-157
10540335-1	1999	Amongst lymphocytes, expression of CD77 (globotriaosylceramide, Gb3) is exclusive to B cells of the germinal center (GC).	globotriaosylceramide	41-62	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	35-39
10479149-6	1999	TNF-alpha and IL-1beta treatment was associated with the increased HBMEC expression of the toxin-binding glycolipid globotriaosylceramide.	globotriaosylceramide	116-137	tumor necrosis factor	Homo sapiens	0-9
10479149-6	1999	TNF-alpha and IL-1beta treatment was associated with the increased HBMEC expression of the toxin-binding glycolipid globotriaosylceramide.	globotriaosylceramide	116-137	interleukin 1 beta	Homo sapiens	14-22
10233996-3	1999	We identified globotriaosylceramide (Gb3) and ganglioside GM3 as the main glycosphingolipids recognized by gp120.	globotriaosylceramide	14-35	inter-alpha-trypsin inhibitor heavy chain 4	Homo sapiens	107-112
10198223-1	1999	The globotriaosylceramide (Gb3) verotoxin (VT) interaction is one of several examples of glycolipid receptors where the ceramide (or lipid) free oligosaccharides fail to show the expected binding parameters.	globotriaosylceramide	4-25	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	27-30
9988695-5	1999	Whereas [3H]globotriaosylceramide ([3H]Gb3) comprised 1.9% of the total [3H]SLs and [3H]GSLs synthesized in control cells, it comprised 16.	globotriaosylceramide	12-33	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	39-42
9826718-0	1998	The neutral glycosphingolipid globotriaosylceramide promotes fusion mediated by a CD4-dependent CXCR4-utilizing HIV type 1 envelope glycoprotein.	globotriaosylceramide	30-51	CD4 molecule	Homo sapiens	82-85
9826718-0	1998	The neutral glycosphingolipid globotriaosylceramide promotes fusion mediated by a CD4-dependent CXCR4-utilizing HIV type 1 envelope glycoprotein.	globotriaosylceramide	30-51	C-X-C motif chemokine receptor 4	Homo sapiens	96-101
9507533-1	1997	The presence of cell surface receptor glycolipid, globotriaosylceramide (Gb3), is essential to confer susceptibility to the E. coli-derived verotoxin (VT).	globotriaosylceramide	50-71	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	73-76
9712788-3	1998	By HPTLC, Shiga toxin was shown to bind globotriaosylceramide (Gb3) and a minor platelet glycolipid with an Rf of 0.03, band 0.03.	globotriaosylceramide	40-61	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	63-66
9648915-1	1998	A region of the N-terminal extracellular domain of the B-cell restricted cell differentiation antigen, CD19, has high amino acid sequence similarity to the receptor binding subunit B of verotoxin 1 (VT), an Escherichia coli elaborated cytotoxin, which specifically binds to the cell surface glycolipid, globotriaosylceramide, also known as the germinal center (GC) B-cell differentiation antigen, CD77.	globotriaosylceramide	303-324	CD19 molecule	Homo sapiens	103-107
9485303-3	1998	The B-pentamer binds to a specific glycolipid, globotriaosylceramide (Gb3), on the surface of target cells and thereby plays a crucial role in the entry of the toxin.	globotriaosylceramide	47-68	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	70-73
9485304-1	1998	The wild-type binding pentamer of Shiga-like toxin IIe (SLT-IIe) binds both the globotriaosylceramide (Gb3) and globotetraosylceramide (Gb4) cell surface glycolipids, whereas the double mutant GT3 (Q65E/K67Q) exhibits a marked preference for Gb3 [Tyrrell, G. J., et al.	globotriaosylceramide	80-101	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	103-106
9821866-0	1998	Globotriaosyl ceramide (Gb3) expression in human tumour cells: intracellular trafficking defines a new retrograde transport pathway from the cell surface to the nucleus, which correlates with sensitivity to verotoxin.	globotriaosylceramide	0-22	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	24-27
9803963-3	1998	Compared with PBMC from seronegative donors, the GSL metabolism in PBMC from HIV-1-infected individuals was characterized by an increased synthesis of two GSL: the B-lymphocyte differentiation antigen globotriaosylceramide (Gb3, also referred to as CD77), and the monosialoganglioside GM3, a marker of T-lymphocytes and macrophages.	globotriaosylceramide	201-222	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	224-227
9402090-3	1997	The functional receptor for these closely related toxins appears to be a glycosphingolipid, globotriaosylceramide (Gb3).	globotriaosylceramide	92-113	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	115-118
9214299-3	1997	Protons of the exocyclic hydroxymethyl group on the terminal Gal residue of globotriaosylceramide (Gb3) were replaced with deuterium.	globotriaosylceramide	76-97	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	99-102
9083293-11	1997	Thin layer chromatography of extracted glycolipids from the GMVEC showed binding of VT-1 to globotriaosylceramide (Gb3), known to be the functional receptor for VT.	globotriaosylceramide	92-113	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	115-118
7599789-0	1995	Expression of the glycolipid globotriaosylceramide (Gb3) in testicular carcinoma in situ.	globotriaosylceramide	29-50	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	52-55
8827457-3	1996	To explore the functional significance of the change, we examined the role of neutral glycosphingolipids as receptors for the murine pulmonary surfactant protein, SP-A, and found that SP-A bound to LacCer, Gg3Cer, and Gg1Cer, but not to Gb3Cer, Gb4Cer, IV3GalNAc alpha-Gb4Cer, sulfatide, or several gangliosides.	globotriaosylceramide	237-243	surfactant associated protein A1	Mus musculus	184-188
7577818-1	1995	Possible binding sites for the glycolipid globotriaosylceramide (Gal alpha 1-->4Gal beta 1-->4Glc beta 1-->1 Cer; Gb3) on the B-subunits of verotoxin-1 (VT1) were explored using binding data for specifically mutated verotoxins and by computational docking of favoured conformers of Gb3 with the crystal structure of VT1.	globotriaosylceramide	42-63	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	123-126
7577818-1	1995	Possible binding sites for the glycolipid globotriaosylceramide (Gal alpha 1-->4Gal beta 1-->4Glc beta 1-->1 Cer; Gb3) on the B-subunits of verotoxin-1 (VT1) were explored using binding data for specifically mutated verotoxins and by computational docking of favoured conformers of Gb3 with the crystal structure of VT1.	globotriaosylceramide	42-63	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	291-294
7530504-8	1995	To examine the role of the two recently cloned TNF-receptors (TNFR-p75 and TNFR-p55) in the TNF alpha-induced increase in GbOse3cer in human endothelial cells, cells were incubated with TNF alpha, the TNFR-p55 selective R32W-S86T-TNF alpha-mutant, or the TNFR-p75 selective D143N-A145R-TNF alpha-mutant.	globotriaosylceramide	122-131	tumor necrosis factor receptor superfamily, member 1a	Mus musculus	62-66
7530504-8	1995	To examine the role of the two recently cloned TNF-receptors (TNFR-p75 and TNFR-p55) in the TNF alpha-induced increase in GbOse3cer in human endothelial cells, cells were incubated with TNF alpha, the TNFR-p55 selective R32W-S86T-TNF alpha-mutant, or the TNFR-p75 selective D143N-A145R-TNF alpha-mutant.	globotriaosylceramide	122-131	tumor necrosis factor receptor superfamily, member 1a	Mus musculus	75-79
7530504-8	1995	To examine the role of the two recently cloned TNF-receptors (TNFR-p75 and TNFR-p55) in the TNF alpha-induced increase in GbOse3cer in human endothelial cells, cells were incubated with TNF alpha, the TNFR-p55 selective R32W-S86T-TNF alpha-mutant, or the TNFR-p75 selective D143N-A145R-TNF alpha-mutant.	globotriaosylceramide	122-131	tumor necrosis factor	Homo sapiens	92-101
7530504-8	1995	To examine the role of the two recently cloned TNF-receptors (TNFR-p75 and TNFR-p55) in the TNF alpha-induced increase in GbOse3cer in human endothelial cells, cells were incubated with TNF alpha, the TNFR-p55 selective R32W-S86T-TNF alpha-mutant, or the TNFR-p75 selective D143N-A145R-TNF alpha-mutant.	globotriaosylceramide	122-131	TNF receptor superfamily member 1A	Homo sapiens	75-79
7530504-8	1995	To examine the role of the two recently cloned TNF-receptors (TNFR-p75 and TNFR-p55) in the TNF alpha-induced increase in GbOse3cer in human endothelial cells, cells were incubated with TNF alpha, the TNFR-p55 selective R32W-S86T-TNF alpha-mutant, or the TNFR-p75 selective D143N-A145R-TNF alpha-mutant.	globotriaosylceramide	122-131	TNF receptor superfamily member 1A	Homo sapiens	75-79
8902189-2	1996	We have previously reported light microscopic pleiomorphic changes in cells suggestive of altered plasma membranes, an increase in globotriaosylceramide (Gb3), reflected by an increase in Gb3 on the surface of the plasma membrane, and a decrease in the rate and amount of ganglioside synthesized.	globotriaosylceramide	131-152	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	154-157
8807854-1	1996	BACKGROUND: The Escherichia coli verotoxins (VTs) can initiate human vascular disease via the specific recognition of globotriaosyl-ceramide (Gb3) on target endothelial cells.	globotriaosylceramide	118-140	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	142-145
7657808-0	1995	Maturational regulation of globotriaosylceramide, the Shiga-like toxin 1 receptor, in cultured human gut epithelial cells.	globotriaosylceramide	27-48	unconventional SNARE in the ER 1	Homo sapiens	54-72
7868240-0	1995	Globotriaosylceramide, Gb3, is an alternative functional receptor for Shiga-like toxin 2e.	globotriaosylceramide	0-21	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	23-26
7599789-3	1995	In this study, the expression of the globo-series core-structure, globotriaosylceramide (Gb3) was investigated in the preinvasive stage of testicular germ cell tumours, carcinoma in situ (CIS).	globotriaosylceramide	66-87	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	89-92
7516406-0	1994	CD19 has a potential CD77 (globotriaosyl ceramide)-binding site with sequence similarity to verotoxin B-subunits: implications of molecular mimicry for B cell adhesion and enterohemorrhagic Escherichia coli pathogenesis.	globotriaosylceramide	27-49	CD19 molecule	Homo sapiens	0-4
7516406-0	1994	CD19 has a potential CD77 (globotriaosyl ceramide)-binding site with sequence similarity to verotoxin B-subunits: implications of molecular mimicry for B cell adhesion and enterohemorrhagic Escherichia coli pathogenesis.	globotriaosylceramide	27-49	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	21-25
7516406-1	1994	The glycosphingolipid globotriaosyl ceramide (CD77) and other globo-series glycolipids containing terminal galactose (Gal)alpha 1-4Gal residues function as receptors for the verotoxin (Shiga-like toxin) family of Escherichia coli-elaborated toxins.	globotriaosylceramide	22-44	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	46-50
8157640-11	1994	C20:0 and C22:1 containing Gb3 had the greatest capacity to bind VT1.	globotriaosylceramide	27-30	Sp7 transcription factor 7	Mus musculus	10-13
1333300-8	1992	Glycolipid extracts of TNF-alpha-treated cells tested on thin-layer chromatography demonstrated an increase of globotriaosylceramide (GbOse3cer), a functional receptor for VT-1, which suggests that preincubation of human endothelial cells with TNF-alpha leads to an increase in GbOse3cer synthesis in these cells.	globotriaosylceramide	111-132	tumor necrosis factor	Homo sapiens	23-32
8158025-2	1994	Shiga toxin recognizes a galactose-alpha 1-->4-galactose terminal glycolipid, globotriaosylceramide (Gb3), in sensitive mammalian cells and is translocated by endocytosis to the cytoplasm, where it blocks protein synthesis.	globotriaosylceramide	81-102	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	104-107
8250238-1	1993	Galactosyltransferase is required for the addition of galactose to lactosylceramide (galactose beta 1-4 glucose beta 1-1 ceramide), resulting in the synthesis of globotriaosylceramide (Gb3).	globotriaosylceramide	162-183	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	185-188
8250811-10	1993	An increase of globotriaosylceramide (Gb3) was observed, suggesting that that preincubation of endothelial cells with TNF alpha leads to an increase in Gb3 synthesis in these cells.	globotriaosylceramide	15-36	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	38-41
8250811-10	1993	An increase of globotriaosylceramide (Gb3) was observed, suggesting that that preincubation of endothelial cells with TNF alpha leads to an increase in Gb3 synthesis in these cells.	globotriaosylceramide	15-36	tumor necrosis factor	Homo sapiens	118-127
8250811-10	1993	An increase of globotriaosylceramide (Gb3) was observed, suggesting that that preincubation of endothelial cells with TNF alpha leads to an increase in Gb3 synthesis in these cells.	globotriaosylceramide	15-36	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	152-155
1333300-8	1992	Glycolipid extracts of TNF-alpha-treated cells tested on thin-layer chromatography demonstrated an increase of globotriaosylceramide (GbOse3cer), a functional receptor for VT-1, which suggests that preincubation of human endothelial cells with TNF-alpha leads to an increase in GbOse3cer synthesis in these cells.	globotriaosylceramide	134-143	tumor necrosis factor	Homo sapiens	23-32
1333300-8	1992	Glycolipid extracts of TNF-alpha-treated cells tested on thin-layer chromatography demonstrated an increase of globotriaosylceramide (GbOse3cer), a functional receptor for VT-1, which suggests that preincubation of human endothelial cells with TNF-alpha leads to an increase in GbOse3cer synthesis in these cells.	globotriaosylceramide	134-143	tumor necrosis factor	Homo sapiens	244-253
1333300-8	1992	Glycolipid extracts of TNF-alpha-treated cells tested on thin-layer chromatography demonstrated an increase of globotriaosylceramide (GbOse3cer), a functional receptor for VT-1, which suggests that preincubation of human endothelial cells with TNF-alpha leads to an increase in GbOse3cer synthesis in these cells.	globotriaosylceramide	278-287	tumor necrosis factor	Homo sapiens	23-32
1491612-6	1992	Globotriaosylceramide (Gb3) is found at 50 nM and 73 nM for the NFA and HFA species, respectively, while globoside (Gb4) is found at 45 nM and 46 nM for the NFA and HFA species.	globotriaosylceramide	0-21	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	23-26
1305422-4	1992	Binding of these two antibodies to the GM3-lactam-BSA conjugate was inhibited by soluble glycosides of GM2-, GM3-, and GM4-lactam and by GM3- and GM4-lactam, respectively, but not by Gb3 or asialo-GM1 and GM2-saccharides.	globotriaosylceramide	183-186	granulocyte macrophage antigen 3	Mus musculus	39-42
1305422-4	1992	Binding of these two antibodies to the GM3-lactam-BSA conjugate was inhibited by soluble glycosides of GM2-, GM3-, and GM4-lactam and by GM3- and GM4-lactam, respectively, but not by Gb3 or asialo-GM1 and GM2-saccharides.	globotriaosylceramide	183-186	granulocyte macrophage antigen 3	Mus musculus	109-112
1305422-4	1992	Binding of these two antibodies to the GM3-lactam-BSA conjugate was inhibited by soluble glycosides of GM2-, GM3-, and GM4-lactam and by GM3- and GM4-lactam, respectively, but not by Gb3 or asialo-GM1 and GM2-saccharides.	globotriaosylceramide	183-186	granulocyte macrophage antigen 3	Mus musculus	109-112
1305422-4	1992	Binding of these two antibodies to the GM3-lactam-BSA conjugate was inhibited by soluble glycosides of GM2-, GM3-, and GM4-lactam and by GM3- and GM4-lactam, respectively, but not by Gb3 or asialo-GM1 and GM2-saccharides.	globotriaosylceramide	183-186	T cell receptor alpha variable 6-3	Mus musculus	146-149
2078521-1	1990	The neutral glycosphingolipid (GSL) globotriaosylceramide (Gb3) of the globo-series was recently defined as the CD77 antigen.	globotriaosylceramide	36-57	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	59-62
1527419-3	1992	Globotriaosylceramide (Gb3), a glycolipid known to bind to both Shiga toxin and Shiga-like toxins, was found to occur in human milk with an average concentration of 73 nM (77 micrograms/L) for Gb3 containing hydroxylated fatty acids and 50 nM (53 micrograms/L) for Gb3 containing nonhydroxylated fatty acids.	globotriaosylceramide	0-21	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	23-26
1527419-3	1992	Globotriaosylceramide (Gb3), a glycolipid known to bind to both Shiga toxin and Shiga-like toxins, was found to occur in human milk with an average concentration of 73 nM (77 micrograms/L) for Gb3 containing hydroxylated fatty acids and 50 nM (53 micrograms/L) for Gb3 containing nonhydroxylated fatty acids.	globotriaosylceramide	0-21	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	193-196
1527419-3	1992	Globotriaosylceramide (Gb3), a glycolipid known to bind to both Shiga toxin and Shiga-like toxins, was found to occur in human milk with an average concentration of 73 nM (77 micrograms/L) for Gb3 containing hydroxylated fatty acids and 50 nM (53 micrograms/L) for Gb3 containing nonhydroxylated fatty acids.	globotriaosylceramide	0-21	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	193-196
1537890-2	1992	Verotoxin (VT) binds to globotriaosylceramide (Gal alpha 1-4Gal beta 1-4GlcCer Gb3) in susceptible cells.	globotriaosylceramide	24-45	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	79-82
1310610-1	1992	Globotriaosylceramide [(Gal alpha 1-4Gal beta 1-4Glc-ceramide (Gb3)] was separated from human kidney, and the fatty acid composition was determined.	globotriaosylceramide	0-21	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	63-66
1709864-1	1991	We have previously reported that a neutral glycolipid (globotriosylceramide; Gb3) was specifically expressed on Burkitt"s lymphoma cells and on a subset of germinal center tonsillar B lymphocytes.	globotriaosylceramide	55-75	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	77-80
2162261-1	1990	Globotriaosylceramide, the natural substrate of alpha-galactosidase A (the enzyme deficient in Fabry"s disease) was prepared from human kidney by repeated medium pressure chromatography on Lichroprep Si 60 (E. Merck) before and after peracetylation.	globotriaosylceramide	0-21	galactosidase alpha	Homo sapiens	48-69
1314564-2	1992	Previous studies have implicated the glycolipid receptor for the Escherichia coli-derived verotoxin, globotriaosylceramide (Gb3; Gal alpha 1-4Gal beta 1-4Glc-ceramide), in the mechanism of alpha 2 interferon signal transduction.	globotriaosylceramide	101-122	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	124-127
1649877-8	1991	The relative resistance of EC to Shiga toxin and Shiga-like toxins may be due to reduced toxin binding, as low levels of globotriaosylceramide (Gb3), the toxin-specific receptor, were found in EC membranes.	globotriaosylceramide	121-142	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	144-147
1910293-3	1991	Both of these cell lines contain significant levels of the verotoxin binding glycolipid globotriosylceramide (Gb3) (1 nmol/10(7) cells and 3 nmol/10(6) cells, respectively).	globotriaosylceramide	88-108	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	110-113
2200824-5	1990	By contrast, permeabilized mouse IL-3-dependent bone marrow culture-derived mast cells (BMMC) and mast cells recovered after 21 days of coculture of BMMC with mouse 3T3 fibroblasts expressed lactosylceramide, globotriosylceramide, globotetraosylceramide, ganglioside GM1, and ganglioside GM3, but not globopentaosylceramide intracellularly as determined by immunofluorescence.	globotriaosylceramide	209-229	interleukin 3	Mus musculus	33-37
2320574-3	1990	Saposin B, previously known as SAP-1 and sulfatide activator, stimulates the hydrolysis of a wide variety of substrates including cerebroside sulfate, GM1 ganglioside, and globotriaosylceramide by arylsulfatase A, acid beta-galactosidase, and alpha-galactosidase, respectively.	globotriaosylceramide	172-193	prosaposin	Homo sapiens	31-36
2157788-4	1990	Gas-liquid chromatography revealed that globotriaosylceramide (Gb3) was markedly increased in the heart (32.4 times higher than control) and increased to a lesser extent in the liver and kidney (3.74 and 6.79 times, respectively).	globotriaosylceramide	40-61	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	63-66
2078521-1	1990	The neutral glycosphingolipid (GSL) globotriaosylceramide (Gb3) of the globo-series was recently defined as the CD77 antigen.	globotriaosylceramide	36-57	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	112-116
2298824-10	1990	Analysis of the glycosphingolipid (GSL) composition of m5S/1M cells showed that globotriaosylceramide (Gb3Cer), which is known to be a Burkitt lymphoma-associated antigen, is specifically expressed in the EGF-treated cells.	globotriaosylceramide	80-101	epidermal growth factor	Mus musculus	205-208
2298824-10	1990	Analysis of the glycosphingolipid (GSL) composition of m5S/1M cells showed that globotriaosylceramide (Gb3Cer), which is known to be a Burkitt lymphoma-associated antigen, is specifically expressed in the EGF-treated cells.	globotriaosylceramide	103-109	epidermal growth factor	Mus musculus	205-208
34796992-1	2022	Fabry disease (FD), which is a lysosomal storage disease resulting from a deficiency of alpha-galactosidase A, leads to the accumulation of globotriaosylceramide in various tissues and multiorgan impairment.	globotriaosylceramide	140-161	galactosidase alpha	Homo sapiens	88-109
33768330-2	2021	The intracellular deposition globotriaosylceramide (Gb3) is just the first step of the mechanism.	globotriaosylceramide	29-50	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	52-55
11741960-3	2002	Thus, certain neutral glycosphingolipids (e.g. GlcCer, LacCer, and Gb(3)Cer) can enhance anticoagulant activity of APC/protein S by mechanisms that are distinctly different from those of phospholipids alone.	globotriaosylceramide	67-75	APC regulator of WNT signaling pathway	Homo sapiens	115-118
32612933-5	2020	After starting ERT serial urine globotriaosylceramide (Gb3) measurements showed an upward trend from 333 to 2260 mug/mmol creatinine for patient 1 and 1165 to 2260 mug/mmol creatinine for patient 2.	globotriaosylceramide	32-53	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	55-58
34885938-2	2021	As a result, the main glycosphingolipid substrates, globotriaosylceramide (Gb3) and globotriaosylsphingosine (lyso-Gb3), accumulate in plasma, urine, and tissues.	globotriaosylceramide	52-73	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	75-78
34917096-2	2021	Progressive cellular accumulation of the AGAL substrate globotriaosylceramide (Gb3) leads to endothelial dysfunction.	globotriaosylceramide	56-77	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	79-82
34704396-1	2021	AIMS: Fabry disease (FD) is an X-linked genetic disease caused by mutations in the GLA gene that leads to deficient activity of lysosomal enzymes, accumulation of globotriaosylceramide in multi-organ systems, and variant clinical manifestations.	globotriaosylceramide	163-184	galactosidase alpha	Homo sapiens	83-86
34748189-2	2021	The lysosomal accumulation of the substrates globotriaosylceramide (Gb3) and globotriaosylsphingosine (lyso-Gb3) results in progressive renal failure, cardiomyopathy associated with cardiac arrhythmia, and recurrent strokes, significantly limiting life expectancy in affected patients.	globotriaosylceramide	45-66	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	68-71
34803097-1	2021	Fabry disease is a rare X-linked lysosomal storage disorder caused by mutations in the alpha-galactosidase A (GLA) gene that results in deficiency of the enzyme GLA and leads to the accumulation of globotriaosylceramide (GL-3) in cells.	globotriaosylceramide	198-219	galactosidase, alpha	Mus musculus	87-108
34803097-1	2021	Fabry disease is a rare X-linked lysosomal storage disorder caused by mutations in the alpha-galactosidase A (GLA) gene that results in deficiency of the enzyme GLA and leads to the accumulation of globotriaosylceramide (GL-3) in cells.	globotriaosylceramide	198-219	galactosidase, alpha	Mus musculus	110-113
34541380-6	2021	FD myelinated axons exhibited lipid accumulations that were determined to be the FD-associated lipid globotriaosylceramide (Gb3), and to a lesser extent lysosomes.	globotriaosylceramide	101-122	alpha 1,4-galactosyltransferase (P blood group)	Rattus norvegicus	124-127
34631425-1	2021	In Fabry disease, accumulation of glycolipids, predominantly globotriaosylceramide (Gb3), affects the kidneys, and nephropathy is one of the important disorders that influence the disease severity and prognosis of patients.	globotriaosylceramide	61-82	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	84-87
34411438-2	2021	We discovered increased levels of globotriaosylceramide (Gb3) in cellular and murine models of CLN3 and CLN7 diseases and used fluorescent-conjugated bacterial toxins to label Gb3 to develop a cell-based high content imaging (HCI) screening assay for the repurposing of FDA-approved compounds able to reduce this accumulation within BD cells.	globotriaosylceramide	34-55	ceroid lipofuscinosis, neuronal 3, juvenile (Batten, Spielmeyer-Vogt disease)	Mus musculus	95-99
34576250-2	2021	Lysosomal accumulation of globotriaosylceramide (Gb3) is the element characterizing Fabry disease due to a hereditary deficiency alpha-galactosidase A (GLA) enzyme.	globotriaosylceramide	26-47	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	49-52
34422789-5	2021	This FC in vitro model recapitulated several clinical FC features, including cardiomyocyte hypertrophy and lysosomal globotriaosylceramide (Gb3) deposition.	globotriaosylceramide	117-138	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	140-143
34300196-2	2021	Deficiency or reduced activity of alpha-galactosidase A (GLA) is leading to progressive intracellular accumulation of globotriaosylceramide (GL3) in various organs, including the heart, kidney and nerve system.	globotriaosylceramide	118-139	galactosidase alpha	Homo sapiens	57-60
34440358-0	2021	MiRNA Let-7a and Let-7d Are Induced by Globotriaosylceramide via NF-kB Activation in Fabry Disease.	globotriaosylceramide	39-60	microRNA let-7d	Homo sapiens	17-23
34300196-2	2021	Deficiency or reduced activity of alpha-galactosidase A (GLA) is leading to progressive intracellular accumulation of globotriaosylceramide (GL3) in various organs, including the heart, kidney and nerve system.	globotriaosylceramide	118-139	galactosidase alpha	Homo sapiens	34-55
34073185-1	2021	The B subunit pentamer verotoxin (VT aka Shiga toxin-Stx) binding to its cellular glycosphingolipid (GSL) receptor, globotriaosyl ceramide (Gb3) mediates internalization and the subsequent receptor mediated retrograde intracellular traffic of the AB5 subunit holotoxin to the endoplasmic reticulum.	globotriaosylceramide	116-138	ST8 alpha-N-acetyl-neuraminide alpha-2,8-sialyltransferase 2	Homo sapiens	53-56
34155339-2	2022	The glycolipid globotriaosylceramide (Gb3) is the main receptor for Shiga toxin (Stx) in kidney target cells.	globotriaosylceramide	15-36	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	38-41
34204583-2	2021	The absence of the enzyme or its activity results in the accumulation of glycosphingolipids, mainly globotriaosylceramide (Gb3), in different tissues, leading to a wide range of clinical manifestations.	globotriaosylceramide	100-121	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	123-126
34073185-1	2021	The B subunit pentamer verotoxin (VT aka Shiga toxin-Stx) binding to its cellular glycosphingolipid (GSL) receptor, globotriaosyl ceramide (Gb3) mediates internalization and the subsequent receptor mediated retrograde intracellular traffic of the AB5 subunit holotoxin to the endoplasmic reticulum.	globotriaosylceramide	116-138	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	140-143
34284652-1	2021	Fabry disease (FD) is a progressive, X-linked inherited disorder of glycosphingolipid metabolism due to deficient or absent lysosomal alpha-galactosidase A (alpha-Gal A) activity which results in progressive accumulation of globotriaosylceramide (Gb3) and related metabolites.	globotriaosylceramide	224-245	galactosidase alpha	Homo sapiens	134-155
34284652-1	2021	Fabry disease (FD) is a progressive, X-linked inherited disorder of glycosphingolipid metabolism due to deficient or absent lysosomal alpha-galactosidase A (alpha-Gal A) activity which results in progressive accumulation of globotriaosylceramide (Gb3) and related metabolites.	globotriaosylceramide	224-245	galactosidase alpha	Homo sapiens	157-168
34284652-1	2021	Fabry disease (FD) is a progressive, X-linked inherited disorder of glycosphingolipid metabolism due to deficient or absent lysosomal alpha-galactosidase A (alpha-Gal A) activity which results in progressive accumulation of globotriaosylceramide (Gb3) and related metabolites.	globotriaosylceramide	224-245	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	247-250
35512362-1	2022	AIMS: Fabry disease (FD) is an X-linked lysosomal storage disorder caused by a deficiency of the lysosomal enzyme alpha-galactosidase A (GLA/AGAL), resulting in the lysosomal accumulation of globotriaosylceramide (Gb3).	globotriaosylceramide	191-212	galactosidase alpha	Homo sapiens	114-135
34609404-1	2021	Fabry disease represents an X-linked inherited disorder resulting in the accumulation of globotriaosylceramide (Gb3).	globotriaosylceramide	89-110	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	112-115
35512362-1	2022	AIMS: Fabry disease (FD) is an X-linked lysosomal storage disorder caused by a deficiency of the lysosomal enzyme alpha-galactosidase A (GLA/AGAL), resulting in the lysosomal accumulation of globotriaosylceramide (Gb3).	globotriaosylceramide	191-212	galactosidase alpha	Homo sapiens	137-140
35512362-1	2022	AIMS: Fabry disease (FD) is an X-linked lysosomal storage disorder caused by a deficiency of the lysosomal enzyme alpha-galactosidase A (GLA/AGAL), resulting in the lysosomal accumulation of globotriaosylceramide (Gb3).	globotriaosylceramide	191-212	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	214-217
35242579-1	2022	Background: Fabry disease (FD) is a lysosomal storage disorder resulting in systemic accumulation of globotriaosylceramide (Gb3) causing multi-organ dysfunction.	globotriaosylceramide	101-122	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	124-127
35246967-1	2022	BACKGROUND: Fabry disease (FD) is caused by a defect in alpha-galactosidase A gene (GLA) which leads to a progressive accumulation of neutral shingolipids, mainly globotriaosylceramide and its metabolites in several organs.	globotriaosylceramide	163-184	galactosidase alpha	Homo sapiens	56-77
35246967-1	2022	BACKGROUND: Fabry disease (FD) is caused by a defect in alpha-galactosidase A gene (GLA) which leads to a progressive accumulation of neutral shingolipids, mainly globotriaosylceramide and its metabolites in several organs.	globotriaosylceramide	163-184	galactosidase alpha	Homo sapiens	84-87
35059891-9	2022	The a-galactosidase A enzyme activity was normal at 6.03 mumol/mL/h (normal 2.40-17.65 mumol/mL/h), and low expression of globotriaosylceramide (Gb3) was detected in the renal tissue of this patient.	globotriaosylceramide	122-143	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	145-148
2644022-7	1989	The holotoxins bind to cells, via their B subunits, to a specific receptor which is probably the glycolipid, globotriosyl ceramide (Gb3).	globotriaosylceramide	109-130	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	132-135
35163813-2	2022	Reduced activity or deficiency of alpha-galactosidase A (AGAL) leads to escalating storage of intracellular globotriaosylceramide (GL-3) in numerous organs, including the kidneys, heart and nerve system.	globotriaosylceramide	108-129	galactosidase alpha	Homo sapiens	34-55
3514610-3	1986	PA1 cells contain globotriaosylceramide, sialosylgangliotriaosylceramide, sialylated and nonsialylated lacto-N-neotetraosylceramide, and the following glycolipids with a blood group type 1 sequence: (formula; see text) The two former glycolipids, lacto-N-tetraosylceramide and sialosyllacto-N-tetraosylceramide, reacted with monoclonal antibodies, K21 and K4, respectively.	globotriaosylceramide	18-39	PAXIP1 associated glutamate rich protein 1	Homo sapiens	0-3
3827882-0	1986	A triple-binding-domain model explains the specificity of the interaction of a sphingolipid activator protein (SAP-1) with sulphatide, GM1-ganglioside and globotriaosylceramide.	globotriaosylceramide	155-176	prosaposin	Homo sapiens	111-116
3566745-1	1987	The deliberate transfer of globotriaosylceramide (Gb3) expression in mouse lymphoma L5178 cells was achieved by transfection with a cosmid DNA library prepared from human Burkitt lymphoma Ramos cells in which Gb3 was highly expressed.	globotriaosylceramide	27-48	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	50-53
3566745-1	1987	The deliberate transfer of globotriaosylceramide (Gb3) expression in mouse lymphoma L5178 cells was achieved by transfection with a cosmid DNA library prepared from human Burkitt lymphoma Ramos cells in which Gb3 was highly expressed.	globotriaosylceramide	27-48	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	209-212
33753109-3	2021	Generally, the B-subunit of Shiga toxin (STXB) receptor, globotriaosylceramide (Gb3), is expressed in high amounts on a number of human tumors cancer cells.	globotriaosylceramide	57-78	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	80-83
3519828-3	1986	A glycolipid that specifically binds shigella toxin was isolated from both HeLa cells and rabbit jejunal mucosa and identified as globotriaosylceramide (Gb3) by its identical mobility on HPTLC to authentic erythrocyte Gb3.	globotriaosylceramide	130-151	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	153-156
3519828-3	1986	A glycolipid that specifically binds shigella toxin was isolated from both HeLa cells and rabbit jejunal mucosa and identified as globotriaosylceramide (Gb3) by its identical mobility on HPTLC to authentic erythrocyte Gb3.	globotriaosylceramide	130-151	alpha 1,4-galactosyltransferase (P blood group)	Homo sapiens	218-221
3081038-2	1986	SAP-1 or sulfatide/GM1 ganglioside activator protein has previously been demonstrated to stimulate the enzymatic hydrolysis of sulfatide, GM1 ganglioside and globotriaosylceramide.	globotriaosylceramide	158-179	prosaposin	Homo sapiens	0-5
33928440-1	2022	The accumulation of globotriaosylceramide (Gb-3) in multiple organs, such as the heart, kidney, and nervous system, due to mutations in the galactosidase alpha (GLA) gene, represents the key point of Fabry disease (FD).	globotriaosylceramide	20-41	galactosidase alpha	Homo sapiens	140-159
33928440-1	2022	The accumulation of globotriaosylceramide (Gb-3) in multiple organs, such as the heart, kidney, and nervous system, due to mutations in the galactosidase alpha (GLA) gene, represents the key point of Fabry disease (FD).	globotriaosylceramide	20-41	galactosidase alpha	Homo sapiens	161-164
33915609-1	2021	OBJECTIVES: Fabry disease (FD) is an X-linked lysosomal storage disorder, resulting from a deficiency of the enzyme alpha-galactosidase A, responsible for breaking down glycolipids such as globotriaosylceramide and its deacylated derivative, globotriaosylsphingosine (LysoGb3).	globotriaosylceramide	189-210	galactosidase alpha	Homo sapiens	116-137