Ibiglustat

产品编号 T4473Cas号 1401090-53-6

别名 Venglustat|||SAR402671|||GZ402671

Ibiglustat (GZ402671) 是一种可透过血脑屏障的、具有口服活性的葡萄糖神经酰胺合成酶(GCS)抑制剂。它可用于戈谢病 3 型、法布瑞氏症、与 GBA 突变相关的帕金森病、GM2 神经节苷脂病和常染色体显性多囊肾病的研究。

产品编号 T4473别名 Venglustat, SAR402671, GZ402671Cas号 1401090-53-6

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生物活性

产品描述	Ibiglustat (GZ402671) is a potent and selective Glucosylceramide synthase inhibitor and ceramide glucosyltransferase inhibitor. Ibiglustat blocks the formation of glucosylceramide (GL-1), a key intermediate in the synthesis of GL-3. Ibiglustat is potentially useful for treating Fabry disease. Fabry disease is a rare lysosomal storage disorder, which results in abnormal tissue deposits of a particular fatty substance called globotriaosylceramide (GL-3 or Gb3) throughout the body.
体外活性	Ibiglustat is in phase II Clinical trials for the treatment of Fabry's disease. Fabry disease, an X-linked disorder of glycosphingolipids that is caused by mutations of the GLA gene that codes for α-galactosidase A, leads to dysfunction of many cell types and includes a systemic vasculopathy [1].
别名	Venglustat, SAR402671, GZ402671

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抗原信息

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化学信息

储存&溶解度

存储

Powder: -20°C for 3 years | In solvent: -80°C for 1 year | Shipping with blue ice.

溶解度信息

DMSO: 50 mg/mL (128.37 mM)

溶液配制表

DMSO