ChemicalBook CAS数据库列表 N -(正丁基)脱氧半乳糖霉素
N -(正丁基)脱氧半乳糖霉素
- CAS号:141206-42-0
- 英文名:N-Butyl-1-deoxygalactonojirimycin
- 中文名:N -(正丁基)脱氧半乳糖霉素
- CBNumber:CB21232075
- 分子式:C10H21NO4
- 分子量:219.28
- MOL File:141206-42-0.mol
N -(正丁基)脱氧半乳糖霉素化学性质
- 熔点 :123-124°C
- 沸点 :394.7±42.0 °C(Predicted)
- 密度 :1.234±0.06 g/cm3(Predicted)
- 储存条件 :Hygroscopic, -20°C Freezer, Under Inert Atmosphere
- 溶解度 :DMF: 20 mg/mL; DMSO: 30 mg/mL; Ethanol: 5 mg/mL; PBS (pH 7.2): 10 mg/mL
- 形态 :A crystalline solid
- 酸度系数(pKa) :13.72±0.70(Predicted)
- 颜色 :White to yellow
- 稳定性 :Hygroscopic
安全信息
- 海关编码 :2933399990
N -(正丁基)脱氧半乳糖霉素性质、用途与生产工艺
- 生物活性 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。
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体外研究
Fabry patient-derived fibroblasts with the genotypes R301G (residual -GalA activity; 20%) R220X (<3%) and W162X (<1%).
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
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.
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N -(正丁基)脱氧半乳糖霉素生产厂家
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