PMID-sentid	Pub_year	Sent_text	compound_name	comp_offset	prot_official_name	organism	prot_offset
18753929-1	2008	OBJECTIVE: N-Butyldeoxynojirimycin (NB-DNJ), an inhibitor of HIV gp120 folding, was assessed as a broadly active therapy for the treatment of HIV/AIDS.	miglustat	36-42	inter-alpha-trypsin inhibitor heavy chain 4	Homo sapiens	65-70
19131642-3	2009	Miglustat (N-butyldeoxynojirimycin, Zavesca), an inhibitor of the alpha-1,2 glucosidase, has been proposed for clinical use in CF because of its effect as a corrector of the defective trafficking of F508del-CFTR.	miglustat	11-34	CF transmembrane conductance regulator	Homo sapiens	207-211
19299496-1	2009	RATIONALE: N-butyldeoxynojyrimicin (NB-DNJ, miglustat [Zavesca]) an approved drug for treating Gaucher disease, was reported to be able to correct the defective trafficking of the F508del-CFTR protein.	miglustat	36-42	cystic fibrosis transmembrane conductance regulator	Mus musculus	188-192
19293774-3	2009	We showed that coincubation of Pompe fibroblasts with recombinant human alpha-glucosidase and the chaperone N-butyldeoxynojirimycin (NB-DNJ) resulted in more efficient correction of enzyme activity.	miglustat	108-131	sucrase-isomaltase	Homo sapiens	72-89
19293774-3	2009	We showed that coincubation of Pompe fibroblasts with recombinant human alpha-glucosidase and the chaperone N-butyldeoxynojirimycin (NB-DNJ) resulted in more efficient correction of enzyme activity.	miglustat	133-139	sucrase-isomaltase	Homo sapiens	72-89
18684516-3	2008	In this study, we suppressed ganglioside biosynthesis in PC12 cells with the glucosylceramide synthase inhibitor, N-butyldeoxynojirimycin and observed reduced plasma membrane antibody binding and a major neuroprotective effect in complement-mediated lysis assays.	miglustat	114-137	UDP-glucose ceramide glucosyltransferase	Rattus norvegicus	77-102
19750228-3	2009	During a combination treatment trial using two such compounds, N-butyldeoxynojirimycin (NB-DNJ) and allopregnanolone, we noted increased lifespan for Npc1(-/-) mice receiving only 2-hydroxypropyl-beta-cyclodextrin (CD), the vehicle for allopregnanolone.	miglustat	63-86	NPC intracellular cholesterol transporter 1	Mus musculus	150-154
19750228-3	2009	During a combination treatment trial using two such compounds, N-butyldeoxynojirimycin (NB-DNJ) and allopregnanolone, we noted increased lifespan for Npc1(-/-) mice receiving only 2-hydroxypropyl-beta-cyclodextrin (CD), the vehicle for allopregnanolone.	miglustat	88-94	NPC intracellular cholesterol transporter 1	Mus musculus	150-154
18753929-1	2008	OBJECTIVE: N-Butyldeoxynojirimycin (NB-DNJ), an inhibitor of HIV gp120 folding, was assessed as a broadly active therapy for the treatment of HIV/AIDS.	miglustat	11-34	inter-alpha-trypsin inhibitor heavy chain 4	Homo sapiens	65-70
17630779-6	2007	The binding of mAb 5F1 to ACE is carbohydrate-dependent and increased significantly as a result of altered glycosylation after treatment with alpha-glucosidase-1 inhibitor, N-butyldeoxynojirimycin (NB-DNJ), or neuraminidase.	miglustat	173-196	angiotensin I converting enzyme	Homo sapiens	26-29
18287616-9	2008	Inhibition of GM3 biosynthesis with the glucosyl transferase inhibitor, N-butyldeoxynojirimycin (NB-DNJ), increased HUVEC proliferation and the phosphorylation of VEGFR-2 and Akt.	miglustat	72-95	kinase insert domain receptor	Homo sapiens	163-170
18287616-9	2008	Inhibition of GM3 biosynthesis with the glucosyl transferase inhibitor, N-butyldeoxynojirimycin (NB-DNJ), increased HUVEC proliferation and the phosphorylation of VEGFR-2 and Akt.	miglustat	72-95	AKT serine/threonine kinase 1	Homo sapiens	175-178
18287616-9	2008	Inhibition of GM3 biosynthesis with the glucosyl transferase inhibitor, N-butyldeoxynojirimycin (NB-DNJ), increased HUVEC proliferation and the phosphorylation of VEGFR-2 and Akt.	miglustat	97-103	kinase insert domain receptor	Homo sapiens	163-170
18287616-9	2008	Inhibition of GM3 biosynthesis with the glucosyl transferase inhibitor, N-butyldeoxynojirimycin (NB-DNJ), increased HUVEC proliferation and the phosphorylation of VEGFR-2 and Akt.	miglustat	97-103	AKT serine/threonine kinase 1	Homo sapiens	175-178
18387328-4	2008	The imino sugars N-butyldeoxynojirimycin (NB-DNJ, miglustat, Zavesca) and N-butyldeoxygalactonojirimycin (NB-DGJ) used for SRT inhibit glucosylceramide synthase (GlcCerS) that catalyses the first committed step in glycosphingolipid biosynthesis.	miglustat	17-40	UDP-glucose ceramide glucosyltransferase	Mus musculus	135-160
17590432-2	2008	Here, we show in human tracheal gland CF-KM4 cells, that after correction of F508del-CFTR trafficking by miglustat (N-butyldeoxynojirimycin) or low temperature (27 degrees C), the Ca(2+) mobilization is decreased compared to uncorrected cells and becomes identical to the Ca(2+) response observed in non-CF MM39 cells.	miglustat	116-139	CF transmembrane conductance regulator	Homo sapiens	85-89
17630779-6	2007	The binding of mAb 5F1 to ACE is carbohydrate-dependent and increased significantly as a result of altered glycosylation after treatment with alpha-glucosidase-1 inhibitor, N-butyldeoxynojirimycin (NB-DNJ), or neuraminidase.	miglustat	198-204	angiotensin I converting enzyme	Homo sapiens	26-29
17213836-2	2007	We studied the effects of two imino sugars, deoxynojirimycin (DNJ) and N-butyldeoxynojirimycin (NB-DNJ), on residual GAA activity in fibroblasts from eight patients with different forms of Pompe disease (two classic infantile, two non-classic infantile onset, four late-onset forms), and with different mutations of the GAA gene.	miglustat	71-94	alpha glucosidase	Homo sapiens	117-120
17213836-2	2007	We studied the effects of two imino sugars, deoxynojirimycin (DNJ) and N-butyldeoxynojirimycin (NB-DNJ), on residual GAA activity in fibroblasts from eight patients with different forms of Pompe disease (two classic infantile, two non-classic infantile onset, four late-onset forms), and with different mutations of the GAA gene.	miglustat	96-102	alpha glucosidase	Homo sapiens	117-120
16627252-1	2006	In a prospective, open-label study, 25 patients with mild-to-moderate type 1 Gaucher"s disease (GD1) were treated with miglustat (Zavesca), an oral glucosylceramide synthase inhibitor, over 12 months.	miglustat	119-128	UDP-glucose ceramide glucosyltransferase	Homo sapiens	148-173
16547352-3	2006	Two inhibitors of GCS, D,L-threo-1-phenyl-2-hexadecanoylamino-3-pyrrolidino-1-propanol (t-PPPP) and N-butyldeoxynojirimycin (NB-dNJ), very efficiently depleted ganglioside content in two human neuroblastoma cell lines.	miglustat	125-131	UDP-glucose ceramide glucosyltransferase	Homo sapiens	18-21
28182897-2	2007	We studied the effects of two imino sugars, deoxynojirimycin (DNJ) and N-butyldeoxynojirimycin (NB-DNJ), on residual GAA activity in fibroblasts from eight patients with different forms of Pompe disease (two classic infantile, two non-classic infantile onset, four late-onset forms), and with different mutations of the GAA gene.	miglustat	71-94	alpha glucosidase	Homo sapiens	117-120
28182897-2	2007	We studied the effects of two imino sugars, deoxynojirimycin (DNJ) and N-butyldeoxynojirimycin (NB-DNJ), on residual GAA activity in fibroblasts from eight patients with different forms of Pompe disease (two classic infantile, two non-classic infantile onset, four late-onset forms), and with different mutations of the GAA gene.	miglustat	96-102	alpha glucosidase	Homo sapiens	117-120
17624027-1	2007	Miglustat (Zavesca) is a reversible inhibitor of glucosylceramide synthase, which catalyses the first step in the glucosylceramide biosynthetic pathway, and is approved for therapy in patients with type 1 Gaucher disease.	miglustat	11-18	UDP-glucose ceramide glucosyltransferase	Homo sapiens	49-74
17095274-5	2007	In case of Y455F/Y455F and P545L/P545L, N-(n-butyl)deoxynojirimycin (NB-DNJ) restored the transport, maturation and activity of AalphaGlu in a dose dependent manner, while it had no effect on the reference enzyme beta-hexosaminidase.	miglustat	40-67	O-GlcNAcase	Homo sapiens	213-232
17095274-5	2007	In case of Y455F/Y455F and P545L/P545L, N-(n-butyl)deoxynojirimycin (NB-DNJ) restored the transport, maturation and activity of AalphaGlu in a dose dependent manner, while it had no effect on the reference enzyme beta-hexosaminidase.	miglustat	69-75	O-GlcNAcase	Homo sapiens	213-232
11446388-8	2001	The treatment with an inhibitor of ceramide-specific glucosyltransferase, N-butyldeoxynojirimysin (NB-DNJ) markedly reduced the intracytoplasmic granular immunofluorescence for GM2 ganglioside in SPMS9 cells, whereas the amount of filipin-positive granules remained unchanged.	miglustat	99-105	cytochrome b5 domain containing 2	Mus musculus	177-180
16546175-2	2006	We show that the alpha-1,2-glucosidase inhibitor miglustat (N-butyldeoxynojirimycin, NB-DNJ) prevents delF508-CFTR/calnexin interaction and restores cAMP-activated chloride current in epithelial CF cells.	miglustat	60-83	CF transmembrane conductance regulator	Homo sapiens	110-114
16546175-2	2006	We show that the alpha-1,2-glucosidase inhibitor miglustat (N-butyldeoxynojirimycin, NB-DNJ) prevents delF508-CFTR/calnexin interaction and restores cAMP-activated chloride current in epithelial CF cells.	miglustat	60-83	calnexin	Homo sapiens	115-123
16546175-2	2006	We show that the alpha-1,2-glucosidase inhibitor miglustat (N-butyldeoxynojirimycin, NB-DNJ) prevents delF508-CFTR/calnexin interaction and restores cAMP-activated chloride current in epithelial CF cells.	miglustat	85-91	CF transmembrane conductance regulator	Homo sapiens	110-114
16546175-2	2006	We show that the alpha-1,2-glucosidase inhibitor miglustat (N-butyldeoxynojirimycin, NB-DNJ) prevents delF508-CFTR/calnexin interaction and restores cAMP-activated chloride current in epithelial CF cells.	miglustat	85-91	calnexin	Homo sapiens	115-123
14757169-1	2004	The imino sugar N-butyldeoxynojirimycin (NB-DNJ) is a glucose analogue which inhibits the glycoprotein N-glycan processing enzymes alpha-glucosidases I and II and the ceramide glucosyltransferase that catalyses the first step of glycosphingolipid biosynthesis.	miglustat	41-47	UDP-glucose ceramide glucosyltransferase	Homo sapiens	167-195
12803930-5	2003	Substrate reduction with N-butyldeoxynojirimycin (OGT 918) is a harbinger of oral iminosugars for glycolipid storage disorders.	miglustat	25-48	O-linked N-acetylglucosamine (GlcNAc) transferase	Homo sapiens	50-53
12542396-7	2003	Furthermore, a truncated, soluble tACE (tACEDelta36NJ), expressed in the presence of the glucosidase-I inhibitor N -butyldeoxynojirimycin, retained the activity of the native enzyme and yielded crystals belonging to the orthorhombic P2(1)2(1)2(1) space group (cell dimensions, a=56.47 A, b=84.90 A, c=133.99 A, alpha=90 degrees, beta=90 degrees and gamma=90 degrees ).	miglustat	113-137	mannosyl-oligosaccharide glucosidase	Homo sapiens	89-102
12239218-7	2002	Complete disulfide bond formation in the CD1d heavy chain was substantially impaired if the chaperone interactions were blocked by the glucosidase inhibitors castanospermine or N-butyldeoxynojirimycin.	miglustat	177-200	CD1d molecule	Homo sapiens	41-45
11525744-5	2001	To evaluate this possibility, we treated murine and feline NPC models with N-butyldeoxynojirimycin (NB-DNJ), an inhibitor of glucosylceramide synthase, a pivotal enzyme in the early GSL synthetic pathway.	miglustat	75-98	UDP-glucose ceramide glucosyltransferase	Mus musculus	125-150
11525744-5	2001	To evaluate this possibility, we treated murine and feline NPC models with N-butyldeoxynojirimycin (NB-DNJ), an inhibitor of glucosylceramide synthase, a pivotal enzyme in the early GSL synthetic pathway.	miglustat	100-106	UDP-glucose ceramide glucosyltransferase	Mus musculus	125-150
12756243-5	2003	Microsomes from Hexb-/- mouse brain showed a significant reduction in the rate of Ca2+-uptake via the sarco/endoplasmic reticulum Ca2+-ATPase (SERCA), which was prevented by feeding Hexb-/- mice with N-butyldeoxynojirimycin (NB-DNJ), an inhibitor of glycolipid synthesis that reduces GM2 storage.	miglustat	200-223	hexosaminidase B	Mus musculus	16-20
12756243-5	2003	Microsomes from Hexb-/- mouse brain showed a significant reduction in the rate of Ca2+-uptake via the sarco/endoplasmic reticulum Ca2+-ATPase (SERCA), which was prevented by feeding Hexb-/- mice with N-butyldeoxynojirimycin (NB-DNJ), an inhibitor of glycolipid synthesis that reduces GM2 storage.	miglustat	200-223	ATPase, Ca++ transporting, ubiquitous	Mus musculus	102-141
12756243-5	2003	Microsomes from Hexb-/- mouse brain showed a significant reduction in the rate of Ca2+-uptake via the sarco/endoplasmic reticulum Ca2+-ATPase (SERCA), which was prevented by feeding Hexb-/- mice with N-butyldeoxynojirimycin (NB-DNJ), an inhibitor of glycolipid synthesis that reduces GM2 storage.	miglustat	200-223	ATPase, Ca++ transporting, ubiquitous	Mus musculus	143-148
12756243-5	2003	Microsomes from Hexb-/- mouse brain showed a significant reduction in the rate of Ca2+-uptake via the sarco/endoplasmic reticulum Ca2+-ATPase (SERCA), which was prevented by feeding Hexb-/- mice with N-butyldeoxynojirimycin (NB-DNJ), an inhibitor of glycolipid synthesis that reduces GM2 storage.	miglustat	200-223	hexosaminidase B	Mus musculus	182-186
12756243-5	2003	Microsomes from Hexb-/- mouse brain showed a significant reduction in the rate of Ca2+-uptake via the sarco/endoplasmic reticulum Ca2+-ATPase (SERCA), which was prevented by feeding Hexb-/- mice with N-butyldeoxynojirimycin (NB-DNJ), an inhibitor of glycolipid synthesis that reduces GM2 storage.	miglustat	225-231	hexosaminidase B	Mus musculus	16-20
12756243-5	2003	Microsomes from Hexb-/- mouse brain showed a significant reduction in the rate of Ca2+-uptake via the sarco/endoplasmic reticulum Ca2+-ATPase (SERCA), which was prevented by feeding Hexb-/- mice with N-butyldeoxynojirimycin (NB-DNJ), an inhibitor of glycolipid synthesis that reduces GM2 storage.	miglustat	225-231	ATPase, Ca++ transporting, ubiquitous	Mus musculus	102-141
12756243-5	2003	Microsomes from Hexb-/- mouse brain showed a significant reduction in the rate of Ca2+-uptake via the sarco/endoplasmic reticulum Ca2+-ATPase (SERCA), which was prevented by feeding Hexb-/- mice with N-butyldeoxynojirimycin (NB-DNJ), an inhibitor of glycolipid synthesis that reduces GM2 storage.	miglustat	225-231	ATPase, Ca++ transporting, ubiquitous	Mus musculus	143-148
12756243-5	2003	Microsomes from Hexb-/- mouse brain showed a significant reduction in the rate of Ca2+-uptake via the sarco/endoplasmic reticulum Ca2+-ATPase (SERCA), which was prevented by feeding Hexb-/- mice with N-butyldeoxynojirimycin (NB-DNJ), an inhibitor of glycolipid synthesis that reduces GM2 storage.	miglustat	225-231	hexosaminidase B	Mus musculus	182-186
12795591-10	2003	Restricted glycosylation of somatic ACE, obtained by the treatment of CHO-ACE cells with the glucosidase inhibitor N-butyldeoxynojirimycin, significantly increased the rate of basal ACE shedding and altered antibody-induced ACE shedding.	miglustat	115-138	angiotensin I converting enzyme	Homo sapiens	36-39
12795591-10	2003	Restricted glycosylation of somatic ACE, obtained by the treatment of CHO-ACE cells with the glucosidase inhibitor N-butyldeoxynojirimycin, significantly increased the rate of basal ACE shedding and altered antibody-induced ACE shedding.	miglustat	115-138	angiotensin I converting enzyme	Homo sapiens	74-77
12795591-10	2003	Restricted glycosylation of somatic ACE, obtained by the treatment of CHO-ACE cells with the glucosidase inhibitor N-butyldeoxynojirimycin, significantly increased the rate of basal ACE shedding and altered antibody-induced ACE shedding.	miglustat	115-138	angiotensin I converting enzyme	Homo sapiens	74-77
12795591-10	2003	Restricted glycosylation of somatic ACE, obtained by the treatment of CHO-ACE cells with the glucosidase inhibitor N-butyldeoxynojirimycin, significantly increased the rate of basal ACE shedding and altered antibody-induced ACE shedding.	miglustat	115-138	angiotensin I converting enzyme	Homo sapiens	74-77
12692077-7	2003	Furthermore, N-(n-butyl)deoxynojirimycin, a potent and non-toxic GCS inhibitor, had no chemosensitizing effect on wild-type melanoma cells.	miglustat	13-40	UDP-glucose ceramide glucosyltransferase	Mus musculus	65-68
12051746-3	2002	Here we show that the use of pH-sensitive liposomes composed of dioleoylphosphatidylethanolamine and cholesteryl hemisuccinate for the delivery of NB-DNJ reduced the required dose for tyrosinase inhibition by a factor of 1000.	miglustat	147-153	tyrosinase	Mus musculus	184-194
12064906-0	2002	Low-dose N-butyldeoxynojirimycin (OGT 918) for type I Gaucher disease.	miglustat	9-32	O-linked N-acetylglucosamine (GlcNAc) transferase	Homo sapiens	34-37
11574545-5	2001	PAR-4 expression and apoptosis were restored by preincubation of ST2-transfected cells with N-butyl deoxinojirimycin (NB-DNJ) or PD98059, two inhibitors of ganglioside biosynthesis or p42/44 mitogen-activated protein (MAPK) kinase, respectively.	miglustat	118-124	PRKC, apoptosis, WT1, regulator	Mus musculus	0-5
10843204-3	2000	Interestingly, N-stearyl-1-deoxynojirimycin (8) displayed potent and specific affinity for gp120 equal to that of 1, a finding that may shed light on the antiviral activity of N-butyl-1-deoxynojirimycin.	miglustat	176-202	inter-alpha-trypsin inhibitor heavy chain 4	Homo sapiens	91-96
10841515-6	2000	Complications observed with another glucosylceramide synthase inhibitor, N-butyldeoxynojirimycin, including weight loss and acellularity of lymphatic organs, were not observed with D-t-EtDO-P4.	miglustat	73-96	UDP-glucose ceramide glucosyltransferase	Mus musculus	36-61
11013265-6	2000	Results using the inhibitor N-butyldeoxynojirimycin, which depletes cellular complex glycosphingolipids, demonstrates that the cholesterol trafficking defect in NPC1 cells is not caused by ganglioside accumulation.	miglustat	28-51	NPC intracellular cholesterol transporter 1	Homo sapiens	161-165
10891408-7	2000	Furthermore, E2 protein expressed in insect cells in the presence of N-butyldeoxynojirimycin, an inhibitor of terminal glucose residue processing, formed complexes with E1 and bound to CD81-EC2 similarly to untreated protein.	miglustat	69-92	ubiquitin conjugating enzyme E2 B	Homo sapiens	13-23
10891408-7	2000	Furthermore, E2 protein expressed in insect cells in the presence of N-butyldeoxynojirimycin, an inhibitor of terminal glucose residue processing, formed complexes with E1 and bound to CD81-EC2 similarly to untreated protein.	miglustat	69-92	CD81 molecule	Homo sapiens	185-189
10801168-0	2000	Novel oral treatment of Gaucher"s disease with N-butyldeoxynojirimycin (OGT 918) to decrease substrate biosynthesis.	miglustat	47-70	O-linked N-acetylglucosamine (GlcNAc) transferase	Homo sapiens	72-75
1387360-1	1992	The imino sugar, N-butyldeoxynojirimycin, is an inhibitor of the glycoprotein-processing enzyme glucosidase I and exhibits anti-(human immunodeficiency virus) activity in vitro.	miglustat	17-40	mannosyl-oligosaccharide glucosidase	Homo sapiens	96-109
8670066-6	1996	Our data also revealed that the alpha glucosidase I inhibitor N-butyldeoxynojirimycin, although affecting the oligosaccharide composition of gp160, does not impair the processing of either gp160 or gp120 by either furin or PACE4.	miglustat	62-85	glutamyl aminopeptidase	Homo sapiens	141-146
8548334-2	1995	Twenty-nine patients were enrolled in a phase I dose-escalating tolerance trial of N-butyl-deoxynojirimycin, an alpha-glucosidase I inhibitor that inhibits human immunodeficiency virus (HIV)-1 replication by altering glycosylation of gp120.	miglustat	83-107	Envelope surface glycoprotein gp160, precursor	Human immunodeficiency virus 1	234-239
1387360-3	1992	We observed selective modulation of the transferrin receptor in response to treatment with 0.5 mM N-butyldeoxynojirimycin resulting in reduced cell-surface transferrin-receptor expression.	miglustat	98-121	transferrin	Homo sapiens	40-51
1387360-3	1992	We observed selective modulation of the transferrin receptor in response to treatment with 0.5 mM N-butyldeoxynojirimycin resulting in reduced cell-surface transferrin-receptor expression.	miglustat	98-121	transferrin	Homo sapiens	156-167
1387360-5	1992	Pulse/chase analysis in conjunction with endoglycosidase-H digestion demonstrated that transferrin-receptor glycosylation was altered following N-butyldeoxynojirimycin treatment, which is compatible with glucosidase inhibition.	miglustat	144-167	transferrin	Homo sapiens	87-98
1387360-6	1992	In addition, modulation of transferrin receptor in response to N-butyldeoxynojirimycin was not confined to a single cell line, but was also observed with certain human lymphoid and myeloid cell lines.	miglustat	63-86	transferrin	Homo sapiens	27-38
34697142-4	2021	MATERIALS AND METHODS: We analyzed the expression of glycosphingolipids and cell growth in MC3T3-E1 mouse osteoblast cells treated with the GCS inhibitors miglustat, D-PDMP and D-PPMP.	miglustat	155-164	UDP-glucose ceramide glucosyltransferase	Mus musculus	140-143
33598405-4	2021	Case presentation: We describe our experience in a now 17-year-old female with NP-C manifest early in childhood who has been successfully and continuously treated with miglustat and KDT in a palliative care setting for 3y.	miglustat	168-177	NPC intracellular cholesterol transporter 1	Homo sapiens	79-83
26879107-7	2016	RESULTS: The clinically used compounds, 4-phenylbutyric acid (4-PBA), suberoylanilide hydroxamic acid (SAHA) and N-butyldeoxynojirimycin (NB-DNJ) improved p.I661T-ATP8B1 plasma membrane targeting.	miglustat	113-136	ATPase phospholipid transporting 8B1	Homo sapiens	163-169
32397443-5	2020	Starting from the example of the marketed drug NBDNJ (N-butyl deoxynojirimycin), a variety of iminosugars have exhibited the capacity to rescue the trafficking of F508del-CFTR (deletion of F508 residue in the CF transmembrane conductance regulator), either alone or in combination with other correctors.	miglustat	54-78	CF transmembrane conductance regulator	Homo sapiens	171-175
29027843-8	2019	At baseline, VRF+ patients had a lower NBV (1530.9 cm3 vs 1591.2 cm3, p = 0.001), a lower cGMV (628.5 cm3 vs 668.6 cm3, p = 0.002) and WMV (752.2 cm3 vs 783.9 cm3, p = 0.009) than VRF-negative patients.	miglustat	39-42	vascular endothelial growth factor B	Homo sapiens	13-16
28610891-5	2017	Several studies have demonstrated that N-butyldeoxynojirimycin (NB-DNJ, also known as miglustat), an inhibitor of the enzyme glucosylceramide synthase (GCS), successfully delays the onset of motor deficits, improves longevity, and rescues some of the cerebellar abnormalities (e.g., Purkinje cell death) seen in another lysosomal disease known as Niemann-Pick type C (NPC).	miglustat	39-62	UDP-glucose ceramide glucosyltransferase	Mus musculus	125-150
28610891-5	2017	Several studies have demonstrated that N-butyldeoxynojirimycin (NB-DNJ, also known as miglustat), an inhibitor of the enzyme glucosylceramide synthase (GCS), successfully delays the onset of motor deficits, improves longevity, and rescues some of the cerebellar abnormalities (e.g., Purkinje cell death) seen in another lysosomal disease known as Niemann-Pick type C (NPC).	miglustat	39-62	UDP-glucose ceramide glucosyltransferase	Mus musculus	152-155
28610891-5	2017	Several studies have demonstrated that N-butyldeoxynojirimycin (NB-DNJ, also known as miglustat), an inhibitor of the enzyme glucosylceramide synthase (GCS), successfully delays the onset of motor deficits, improves longevity, and rescues some of the cerebellar abnormalities (e.g., Purkinje cell death) seen in another lysosomal disease known as Niemann-Pick type C (NPC).	miglustat	64-70	UDP-glucose ceramide glucosyltransferase	Mus musculus	125-150
28610891-5	2017	Several studies have demonstrated that N-butyldeoxynojirimycin (NB-DNJ, also known as miglustat), an inhibitor of the enzyme glucosylceramide synthase (GCS), successfully delays the onset of motor deficits, improves longevity, and rescues some of the cerebellar abnormalities (e.g., Purkinje cell death) seen in another lysosomal disease known as Niemann-Pick type C (NPC).	miglustat	64-70	UDP-glucose ceramide glucosyltransferase	Mus musculus	152-155
28366632-3	2017	We further show that this ER interaction network changes in both content and abundance upon treatment with kifunensine (kif) and N-butyldeoxynojirimycin (NB-DNJ) which suggests that when interfering with the N-glycan processing pathway, the functional complexes involving EDEM3 adapt to maintain the cellular homeostasis.	miglustat	129-152	ER degradation enhancing alpha-mannosidase like protein 3	Homo sapiens	272-277
28366632-3	2017	We further show that this ER interaction network changes in both content and abundance upon treatment with kifunensine (kif) and N-butyldeoxynojirimycin (NB-DNJ) which suggests that when interfering with the N-glycan processing pathway, the functional complexes involving EDEM3 adapt to maintain the cellular homeostasis.	miglustat	154-160	ER degradation enhancing alpha-mannosidase like protein 3	Homo sapiens	272-277
28103924-5	2017	Substrate reduction therapy with miglustat (N-butyldeoxynojirimycin) inhibits glucosylceramide synthase, which catalyzes the first step in glycosphingolipid synthesis.	miglustat	33-42	UDP-glucose ceramide glucosyltransferase	Homo sapiens	78-103
28103924-5	2017	Substrate reduction therapy with miglustat (N-butyldeoxynojirimycin) inhibits glucosylceramide synthase, which catalyzes the first step in glycosphingolipid synthesis.	miglustat	44-67	UDP-glucose ceramide glucosyltransferase	Homo sapiens	78-103
32779865-4	2020	Substrate reduction therapy with Miglustat, a N-alkylated sugar that inhibits the enzyme glucosylceramide synthase, has been proposed for the treatment of several lysosomal storage disorders such as Gaucher type 1 and Niemann Pick Type C diseases.	miglustat	33-42	UDP-glucose ceramide glucosyltransferase	Homo sapiens	89-114
28975712-0	2017	Structure-Activity Studies of N-Butyl-1-deoxynojirimycin (NB-DNJ) Analogues: Discovery of Potent and Selective Aminocyclopentitol Inhibitors of GBA1 and GBA2.	miglustat	30-56	glucosylceramidase beta	Homo sapiens	144-148
28975712-0	2017	Structure-Activity Studies of N-Butyl-1-deoxynojirimycin (NB-DNJ) Analogues: Discovery of Potent and Selective Aminocyclopentitol Inhibitors of GBA1 and GBA2.	miglustat	30-56	glucosylceramidase beta 2	Homo sapiens	153-157
28975712-0	2017	Structure-Activity Studies of N-Butyl-1-deoxynojirimycin (NB-DNJ) Analogues: Discovery of Potent and Selective Aminocyclopentitol Inhibitors of GBA1 and GBA2.	miglustat	58-64	glucosylceramidase beta	Homo sapiens	144-148
28975712-0	2017	Structure-Activity Studies of N-Butyl-1-deoxynojirimycin (NB-DNJ) Analogues: Discovery of Potent and Selective Aminocyclopentitol Inhibitors of GBA1 and GBA2.	miglustat	58-64	glucosylceramidase beta 2	Homo sapiens	153-157
28975712-1	2017	Analogues of N-butyl-1-deoxynojirimycin (NB-DNJ) were prepared and assayed for inhibition of ceramide-specific glucosyltransferase (CGT), non-lysosomal beta-glucosidase 2 (GBA2) and the lysosomal beta-glucosidase 1 (GBA1).	miglustat	13-39	UDP glycosyltransferase 8	Homo sapiens	93-130
28975712-1	2017	Analogues of N-butyl-1-deoxynojirimycin (NB-DNJ) were prepared and assayed for inhibition of ceramide-specific glucosyltransferase (CGT), non-lysosomal beta-glucosidase 2 (GBA2) and the lysosomal beta-glucosidase 1 (GBA1).	miglustat	13-39	UDP glycosyltransferase 8	Homo sapiens	132-135
28975712-1	2017	Analogues of N-butyl-1-deoxynojirimycin (NB-DNJ) were prepared and assayed for inhibition of ceramide-specific glucosyltransferase (CGT), non-lysosomal beta-glucosidase 2 (GBA2) and the lysosomal beta-glucosidase 1 (GBA1).	miglustat	13-39	glucosylceramidase beta 2	Homo sapiens	172-176
28975712-1	2017	Analogues of N-butyl-1-deoxynojirimycin (NB-DNJ) were prepared and assayed for inhibition of ceramide-specific glucosyltransferase (CGT), non-lysosomal beta-glucosidase 2 (GBA2) and the lysosomal beta-glucosidase 1 (GBA1).	miglustat	13-39	glucosylceramidase beta	Homo sapiens	216-220
28975712-1	2017	Analogues of N-butyl-1-deoxynojirimycin (NB-DNJ) were prepared and assayed for inhibition of ceramide-specific glucosyltransferase (CGT), non-lysosomal beta-glucosidase 2 (GBA2) and the lysosomal beta-glucosidase 1 (GBA1).	miglustat	41-47	UDP glycosyltransferase 8	Homo sapiens	93-130
26771826-9	2016	Inhibition of GSL synthesis with the glucosylceramide synthase (GCS) inhibitor N-butyl-1-deoxynojirimycin prevented U18666A-induced Gpnmb induction and secretion.	miglustat	79-105	cathepsin A	Homo sapiens	14-17
27106513-4	2016	N-butyldeoxynojirimycin, an inhibitor of ceramide glucosyltransferase activity and therefore of ganglioside synthesis, was administered to MPS IIIA mice both prior to maximal GM2 and GM3 accumulation (early treatment) and after the maximum level of ganglioside had accumulated in the brain (late treatment) to determine if behaviour was altered by ganglioside level.	miglustat	0-23	UDP-glucose ceramide glucosyltransferase	Mus musculus	41-69
27106513-4	2016	N-butyldeoxynojirimycin, an inhibitor of ceramide glucosyltransferase activity and therefore of ganglioside synthesis, was administered to MPS IIIA mice both prior to maximal GM2 and GM3 accumulation (early treatment) and after the maximum level of ganglioside had accumulated in the brain (late treatment) to determine if behaviour was altered by ganglioside level.	miglustat	0-23	granulocyte macrophage antigen 3	Mus musculus	183-186
26771826-9	2016	Inhibition of GSL synthesis with the glucosylceramide synthase (GCS) inhibitor N-butyl-1-deoxynojirimycin prevented U18666A-induced Gpnmb induction and secretion.	miglustat	79-105	UDP-glucose ceramide glucosyltransferase	Mus musculus	37-62
26771826-9	2016	Inhibition of GSL synthesis with the glucosylceramide synthase (GCS) inhibitor N-butyl-1-deoxynojirimycin prevented U18666A-induced Gpnmb induction and secretion.	miglustat	79-105	UDP-glucose ceramide glucosyltransferase	Mus musculus	64-67
26771826-9	2016	Inhibition of GSL synthesis with the glucosylceramide synthase (GCS) inhibitor N-butyl-1-deoxynojirimycin prevented U18666A-induced Gpnmb induction and secretion.	miglustat	79-105	glycoprotein (transmembrane) nmb	Mus musculus	132-137
22892202-3	2012	Miglustat N-butyldeoxynojirimycin (NBDNJ), an inhibitor of glucosylceramide synthase, has shown much promise in clinical trials for the treatment of Type I Gaucher disease.	miglustat	35-40	UDP-glucose ceramide glucosyltransferase	Homo sapiens	59-84
25915583-4	2015	Glucosylceramide synthase (GCS) inhibitors, including the clinically approved agent N-butyl-deoxynojirimycin (NB-DNJ), prevented OC development and activation by disrupting RANKL-induced localization of TRAF6 and c-SRC into lipid rafts and preventing nuclear accumulation of transcriptional activator NFATc1.	miglustat	84-108	UDP-glucose ceramide glucosyltransferase	Mus musculus	0-25
25915583-4	2015	Glucosylceramide synthase (GCS) inhibitors, including the clinically approved agent N-butyl-deoxynojirimycin (NB-DNJ), prevented OC development and activation by disrupting RANKL-induced localization of TRAF6 and c-SRC into lipid rafts and preventing nuclear accumulation of transcriptional activator NFATc1.	miglustat	84-108	UDP-glucose ceramide glucosyltransferase	Mus musculus	27-30
25915583-4	2015	Glucosylceramide synthase (GCS) inhibitors, including the clinically approved agent N-butyl-deoxynojirimycin (NB-DNJ), prevented OC development and activation by disrupting RANKL-induced localization of TRAF6 and c-SRC into lipid rafts and preventing nuclear accumulation of transcriptional activator NFATc1.	miglustat	84-108	tumor necrosis factor (ligand) superfamily, member 11	Mus musculus	173-178
25915583-4	2015	Glucosylceramide synthase (GCS) inhibitors, including the clinically approved agent N-butyl-deoxynojirimycin (NB-DNJ), prevented OC development and activation by disrupting RANKL-induced localization of TRAF6 and c-SRC into lipid rafts and preventing nuclear accumulation of transcriptional activator NFATc1.	miglustat	84-108	TNF receptor-associated factor 6	Mus musculus	203-208
25915583-4	2015	Glucosylceramide synthase (GCS) inhibitors, including the clinically approved agent N-butyl-deoxynojirimycin (NB-DNJ), prevented OC development and activation by disrupting RANKL-induced localization of TRAF6 and c-SRC into lipid rafts and preventing nuclear accumulation of transcriptional activator NFATc1.	miglustat	84-108	nuclear factor of activated T cells, cytoplasmic, calcineurin dependent 1	Mus musculus	301-307
24463447-8	2014	Inhibition of GSL biosynthesis in vitro with a clinically approved inhibitor (N-butyldeoxynojirimycin) normalized GSL metabolism, corrected CD4+ T cell signaling and functional defects, and decreased anti-dsDNA antibody production by autologous B cells in SLE patients.	miglustat	78-101	cathepsin A	Homo sapiens	14-17
24463447-8	2014	Inhibition of GSL biosynthesis in vitro with a clinically approved inhibitor (N-butyldeoxynojirimycin) normalized GSL metabolism, corrected CD4+ T cell signaling and functional defects, and decreased anti-dsDNA antibody production by autologous B cells in SLE patients.	miglustat	78-101	cathepsin A	Homo sapiens	114-117
23880767-9	2013	Revised as such, GBA2 activity 1) was optimal at pH 5.5-6.0; 2) accounted for a much higher proportion of detergent-independent membrane-associated beta-glucosidase activity; 3) was more variable among mouse tissues and neuroblastoma and monocyte cell lines; and 4) was more sensitive to inhibition by N-butyldeoxynojirimycin (miglustat, Zavesca ), in comparison with earlier studies.	miglustat	302-325	glucosidase beta 2	Mus musculus	17-21
23880767-9	2013	Revised as such, GBA2 activity 1) was optimal at pH 5.5-6.0; 2) accounted for a much higher proportion of detergent-independent membrane-associated beta-glucosidase activity; 3) was more variable among mouse tissues and neuroblastoma and monocyte cell lines; and 4) was more sensitive to inhibition by N-butyldeoxynojirimycin (miglustat, Zavesca ), in comparison with earlier studies.	miglustat	327-336	glucosidase beta 2	Mus musculus	17-21
23880767-9	2013	Revised as such, GBA2 activity 1) was optimal at pH 5.5-6.0; 2) accounted for a much higher proportion of detergent-independent membrane-associated beta-glucosidase activity; 3) was more variable among mouse tissues and neuroblastoma and monocyte cell lines; and 4) was more sensitive to inhibition by N-butyldeoxynojirimycin (miglustat, Zavesca ), in comparison with earlier studies.	miglustat	338-345	glucosidase beta 2	Mus musculus	17-21
25052852-5	2014	alpha-Glucosidase activity was analyzed by tandem-mass spectrometry in dried blood spots from patients treated with enzyme replacement therapy, either alone or in combination with the chaperone N-butyldeoxynojirimycin given at the time of the enzyme infusion.	miglustat	194-217	sucrase-isomaltase	Homo sapiens	0-17
24038832-2	2013	N-butyl 1-deoxynojirimycin (N-Bu DNJ), a clinical candidate for the treatment of cystic fibrosis, is able to act as a CFTR corrector by overcoming the processing defect of the mutant protein.	miglustat	0-26	CF transmembrane conductance regulator	Homo sapiens	118-122
24038832-2	2013	N-butyl 1-deoxynojirimycin (N-Bu DNJ), a clinical candidate for the treatment of cystic fibrosis, is able to act as a CFTR corrector by overcoming the processing defect of the mutant protein.	miglustat	28-36	CF transmembrane conductance regulator	Homo sapiens	118-122
23144710-8	2012	A subset of NPC1 patients on miglustat, a glycosphingolipid synthesis inhibitor, showed significantly decreased levels of FABP3 compared to patients not on miglustat therapy.	miglustat	29-38	NPC intracellular cholesterol transporter 1	Homo sapiens	12-16
23144710-8	2012	A subset of NPC1 patients on miglustat, a glycosphingolipid synthesis inhibitor, showed significantly decreased levels of FABP3 compared to patients not on miglustat therapy.	miglustat	29-38	fatty acid binding protein 3	Homo sapiens	122-127
23144710-8	2012	A subset of NPC1 patients on miglustat, a glycosphingolipid synthesis inhibitor, showed significantly decreased levels of FABP3 compared to patients not on miglustat therapy.	miglustat	156-165	NPC intracellular cholesterol transporter 1	Homo sapiens	12-16
21738789-7	2011	Similar results were observed with NB-DNJ, another iminosugar-based GCS inhibitor.	miglustat	35-41	UDP-glucose ceramide glucosyltransferase	Mus musculus	68-71
21982629-8	2011	Treatment with N-butyl-deoxynojirimycin (NB-DNJ), which acts as a pharmacological chaperone for certain mutant forms of GAA, led to attenuation of not only ER stress, but also autophagy in patient fibroblasts.	miglustat	15-39	alpha glucosidase	Homo sapiens	120-123
21982629-9	2011	Levels of phosphorylated p38 MAPK observed in patient fibroblasts were decreased after treatment with NB-DNJ.	miglustat	102-108	mitogen-activated protein kinase 14	Homo sapiens	25-28
22027144-2	2011	Recently, we found that mutant GAA in patient fibroblasts carrying c.546G>T mutation is stabilized by treatment with proteasome inhibitor as well as pharmacological chaperon N-butyl-deoxynojirimycin.	miglustat	177-201	alpha glucosidase	Homo sapiens	31-34
20980259-7	2010	Our results suggest a mechanism whereby the binding of N-butyl-deoxynojirimycin helps target correctly folded glucosylceramidase to the lysosome, contributes to binding with saposin C, and explains the initiation of the substrate-enzyme complex.	miglustat	55-79	glucosylceramidase beta	Homo sapiens	110-128
20374428-2	2010	Previous studies showed that caloric restriction, which augments longevity, and N-butyldeoxynojirimycin (NB-DNJ, Miglustat), an imino sugar that hinders the glucosyltransferase catalyzing the first step in glycosphingolipid biosynthesis, both increase longevity and improve motor behavior in the beta-hexosaminidase (Hexb) knockout (-/-) murine model of Sandhoff disease.	miglustat	80-103	O-GlcNAcase	Mus musculus	296-315
20374428-2	2010	Previous studies showed that caloric restriction, which augments longevity, and N-butyldeoxynojirimycin (NB-DNJ, Miglustat), an imino sugar that hinders the glucosyltransferase catalyzing the first step in glycosphingolipid biosynthesis, both increase longevity and improve motor behavior in the beta-hexosaminidase (Hexb) knockout (-/-) murine model of Sandhoff disease.	miglustat	80-103	hexosaminidase B	Mus musculus	317-321
20374428-2	2010	Previous studies showed that caloric restriction, which augments longevity, and N-butyldeoxynojirimycin (NB-DNJ, Miglustat), an imino sugar that hinders the glucosyltransferase catalyzing the first step in glycosphingolipid biosynthesis, both increase longevity and improve motor behavior in the beta-hexosaminidase (Hexb) knockout (-/-) murine model of Sandhoff disease.	miglustat	105-111	O-GlcNAcase	Mus musculus	296-315
20374428-2	2010	Previous studies showed that caloric restriction, which augments longevity, and N-butyldeoxynojirimycin (NB-DNJ, Miglustat), an imino sugar that hinders the glucosyltransferase catalyzing the first step in glycosphingolipid biosynthesis, both increase longevity and improve motor behavior in the beta-hexosaminidase (Hexb) knockout (-/-) murine model of Sandhoff disease.	miglustat	105-111	hexosaminidase B	Mus musculus	317-321