Kifunensine

Historically isolated from Kitasatosporia kifunensee.1 Inhibitor of class I α-mannosidases which inhibit glycoprotein processing. Inhibits human endoplasmic reticulum α-1,2-mannosidase I and Golgi Class I mannosidases IA, IB and IC with Ki values of 130 and 23 nM respectively.2 Inhibition of endoplasmic reticulum α-mannosidase I activity rescues the human α-sarcoglycan R77C mutation suggesting a new pharmacological approach for limb girdle muscular dystrophy type 2D patients carrying mutations that impair α-sarcoglycan trafficking.3 Improves maturation of misfolded proteins.4