Afegostat, aslo known as Isofagomine, and AT2101, was an experimental drug for the treatment of certain forms of Gauchers disease. Isofagomine (IFG) is an acid beta-glucosidase (GCase) active site inhibitor that acts as a pharmacological chaperone. FG inhibits GCase with K(i) ~30 nM for wild-type and mutant enzymes (N370S and V394L). Administration of IFG (30 mg/kg/day) to the mice homozygous for GCase mutations (V394L, D409H, or D409V) led to increased GCase activity in visceral tissues and brain extracts.
Molekulargewicht:
261.2
CAS Nummer:
[1084896-50-3]
Formel:
C8H14F3NO5
Target-Kategorie:
glycosidase|||Others
T71476
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