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Lucerastat
本产品不向个人销售,仅用作科学研究,不用于任何人体实验及非科研性质的动物实验。
Lucerastat图片
CAS NO:141206-42-0
包装与价格:
包装价格(元)
5mg电议
10mg电议
25mg电议

产品名称
NB-DGJ
N-(n-Butyl)deoxygalactonojirimycin
产品介绍
Lucerasta 是Miglustat 的半乳糖形式,是具有口服活性的葡萄糖酰神经酰胺合成酶(GCS)的抑制剂。Lucerastat 有用于Fabry 疾病研究的潜能。
生物活性

Lucerastat, the galactose form of Miglustat, is an orally-available inhibitor ofglucosylceramide synthase (GCS). Lucerastat has the potential for Fabry disease study[1][2].

体外研究
(In Vitro)

Fabry patient-derived fibroblasts with the genotypes R301G (residual -GalA activity; 20%) R220X (<3%) and W162X (<1%).

Cell Viability Assay[2].

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.
体内研究
(In Vivo)

Lucerastat (1200 mg/kg/day food admix), a GCS inhibitor, reduces Gb3 in the absence of residual ?-GalA activity[2].

Animal Model:Fabry mice (Gla-/0and Gla-/-, n = 5 or 6 for each gender)[2].
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.
Clinical Trial
分子量

219.28

性状

Solid

Formula

C10H21NO4

CAS 号

141206-42-0

运输条件

Room temperature in continental US; may vary elsewhere.

储存方式

-20°C, sealed storage, away from moisture

*In solvent : -80°C, 6 months; -20°C, 1 month (sealed storage, away from moisture)

溶解性数据
In Vitro: 

H2O : 24 mg/mL(109.45 mM;ultrasonic and warming and heat to 80℃)

DMSO : 22 mg/mL(100.33 mM;ultrasonic and warming and heat to 80℃)

配制储备液
浓度溶剂体积质量1 mg5 mg10 mg
1 mM4.5604 mL22.8019 mL45.6038 mL
5 mM0.9121 mL4.5604 mL9.1208 mL
10 mM0.4560 mL2.2802 mL4.5604 mL
*

请根据产品在不同溶剂中的溶解度选择合适的溶剂配制储备液;一旦配成溶液,请分装保存,避免反复冻融造成的产品失效
储备液的保存方式和期限:-80℃, 6 months; -20℃, 1 month (sealed storage, away from moisture)。-80℃ 储存时,请在 6 个月内使用,-20℃ 储存时,请在 1 个月内使用。