Ibiglustat (Venglustat) succinate is an orally active, brain-penetrant glucosylceramide synthase (GCS) inhibitor. Ibiglustat succinate can be used for the research of Gaucher disease type 3, Parkinson's disease associated with GBA mutations, Fabry disease, GM2 gangliosidosis, and autosomal dominant polycystic kidney disease.
In Vitro
Ibiglustat (SAR402671) succinate (1 μM, 15 days; Fabry disease (FD) cells) is close to the physiological level in untreated WT cells in GL-3 levels, suggesting that Ibiglustat succinate can prevent additional GL-3 accumulation and could serve to ameliorate the abundant levels of this sphingolipid in FD cardiomyocytes . MCE has not independently confirmed the accuracy of these methods. They are for reference only.
Form:Solid
规格
别名
琥珀酸伊比鲁司他
规格或纯度
≥99%
英文名称
Ibiglustat succinate
生化机理
琥珀酸依必鲁司特(文格鲁司特)是一种口服活性脑渗透性葡萄糖酰胺合成酶(GCS)抑制剂。琥珀酸依必鲁司特可用于研究戈谢病 3 型、与 GBA 基因突变相关的帕金森病、法布里病等疾病。