PMID-sentid Pub_year Sent_text compound_name comp_offset prot_official_name organism prot_offset 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 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 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 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 173-196 angiotensin I converting enzyme Homo sapiens 26-29 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 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 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 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 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 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 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 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 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 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 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 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 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 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 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 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 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 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 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 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 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 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 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 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 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 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 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 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 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 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 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 cathepsin A Homo sapiens 14-17 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 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 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 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 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 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 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 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 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 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 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 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 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 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 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 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 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 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 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