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产品名称:米格鲁他

产品别名:

CAS号:72599-27-0

英文别名:米格鲁他

分子量:255.74

分子式:C₁₀H₂₁NO₄?HCl

纯度:98%

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保存方法:Store at -20° C

描述:N-Butyldeoxynojirimycin·HCl is a novel inhibitor of UGCG (glucosylceramide synthase) as well as Glucosidase I and II (α-glucosidase I and II). The compound has been shown to inhibit glucosylceramide synthase without ceramide accumulation and cell death. When administered to young mice the compound reduced glycosphingolipid production 50%-70% in the liver and lymphoid organs without any overt pathology. It is a potent inhibitor of HIV virus replication and syncytium formation in vitro. The compound has been observed to restore cAMP-activated chloride currents in epithelial cystic fibrosis cells through prevented interaction among calnexin and delF508-CFTR. In a mice model for Tay Sachs disease, mice exposed to N-Butyldeoxynojirimycin·HCl limited the biosynthesis of GM2 thus preventing accumulation of GM2 in the brain and glycosphingolipids. This action markedly reduced the number of storage neurons and gangliosides per cell. N-Butyldeoxynojirimycin·HCl has also rescued trafficking-deficient F508del-CTFR in human airway epithelial cells via inhibition of ER-glucosidases I and II. Also has broad spectrum antiviral activity.

应用:Inhibits glucosylceramide synthase and α-glucosidase I and II without overt pathology

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