Lucerastat NB-DGJ; N-(n-Butyldeoxygalactonojirimycin)|cas:141206-42-0|西域试剂

Lucerastat NB-DGJ; N-(n-Butyldeoxygalactonojirimycin)

产品编号：Bellancom-106392| CAS NO：141206-42-0| 分子式：C₁₀H₂₁NO₄| 分子量：219.28

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货号	价格	库存与货期
Bellancom-106392	￥2000.00	杭州北京（现货）
Bellancom-106392	￥3500.00	杭州北京（现货）
Bellancom-106392	￥7350.00	杭州北京（现货）
Bellancom-106392	￥12500.00	杭州北京（现货）

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Lucerastat NB-DGJ; N-(n-Butyldeoxygalactonojirimycin)

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 of glucosylceramide synthase (GCS). Lucerastat has the potential for Fabry disease study.

体外研究

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

西域 has not independently confirmed the accuracy of these methods. They are for reference only.

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.

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.

西域 has not independently confirmed the accuracy of these methods. They are for reference only.

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.

体内研究

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

西域 has not independently confirmed the accuracy of these methods. They are for reference only.

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.

体内研究

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

西域 has not independently confirmed the accuracy of these methods. They are for reference only.

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.

性状

Solid

溶解性数据

In Vitro:

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

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

配制储备液

浓度溶剂体积质量	1 mg	5 mg	10 mg

1 mM	4.5604 mL	22.8019 mL	45.6038 mL
5 mM	0.9121 mL	4.5604 mL	9.1208 mL
10 mM	0.4560 mL	2.2802 mL	4.5604 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°C, 6 months; -20°C, 1 month (sealed storage, away from moisture)。-80°C 储存时，请在 6 个月内使用，-20°C 储存时，请在 1 个月内使用。

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)

参考文献