产品
编 号：F153437
分子式：C10H21NO4
分子量：219.28
分子式：C10H21NO4
分子量：219.28
产品类型
规格
价格
是否有货
1mg
询价
询价
5mg
询价
询价
10mg
询价
询价
25mg
询价
询价
结构图
CAS No: 141206-42-0
产品详情
生物活性：
Lucerastat, the galactose form of Miglustat, is an orally-available inhibitor of glucosylceramide synthase (GCS). Lucerastat has the potential for Fabry disease study.
体内研究：
Lucerastat (1200 mg/kg/day food admix), a GCS inhibitor, reduces Gb3 in the absence of residual-GalA activity.Animal Model:Fabry mice (Gla-/0 and Gla-/-, n = 5 or 6 for each gender).
Dosage:1200 mg/kg/day food admix.
Administration:Food admix for 20 weeks.
Result:Reduced lipid storage in two major organs affected by FD: mean Gb3 in the kidneys (-33%, p<0.01). and α-galactose- terminated glycosphingolipids in the dorsal root ganglia (-48%, p<0.05). In the liver of the Fabry mice, mean glucosylceramide (GlcCer (24:0)) was reduced (-59%, p<0.001) in addition to Gb3 (24:1) (-37%, p<0.05) demonstrating substrate reduction through GCS inhibition.
体外研究：
Fabry patient-derived fibroblasts with the genotypes R301G (residual -GalA activity; 20%) R220X (Lucerastat 相关抗体:Cell Viability Assay.Cell Line:Fabry patient-derived fibroblasts with the genotypes R301G (residual-GalA activity; 20%) R220X (<3%) and W162X (<1%).
Concentration:
Incubation Time:9 days.
Result:Dose-dependently inhibited GCS, reducing glucosylceramide and increasing sphingomyelin.
Lucerastat, the galactose form of Miglustat, is an orally-available inhibitor of glucosylceramide synthase (GCS). Lucerastat has the potential for Fabry disease study.
体内研究：
Lucerastat (1200 mg/kg/day food admix), a GCS inhibitor, reduces Gb3 in the absence of residual-GalA activity.Animal Model:Fabry mice (Gla-/0 and Gla-/-, n = 5 or 6 for each gender).
Dosage:1200 mg/kg/day food admix.
Administration:Food admix for 20 weeks.
Result:Reduced lipid storage in two major organs affected by FD: mean Gb3 in the kidneys (-33%, p<0.01). and α-galactose- terminated glycosphingolipids in the dorsal root ganglia (-48%, p<0.05). In the liver of the Fabry mice, mean glucosylceramide (GlcCer (24:0)) was reduced (-59%, p<0.001) in addition to Gb3 (24:1) (-37%, p<0.05) demonstrating substrate reduction through GCS inhibition.
体外研究：
Fabry patient-derived fibroblasts with the genotypes R301G (residual -GalA activity; 20%) R220X (Lucerastat 相关抗体:Cell Viability Assay.Cell Line:Fabry patient-derived fibroblasts with the genotypes R301G (residual-GalA activity; 20%) R220X (<3%) and W162X (<1%).
Concentration:
Incubation Time:9 days.
Result:Dose-dependently inhibited GCS, reducing glucosylceramide and increasing sphingomyelin.
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