Kifunensine is historically isolated from Kitasatosporia kifunense. It acts as an 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.
Inhibition of endoplasmic reticulum a-mannosidase I activity rescues the human a-sarcoglycan R77C mutation suggesting a new pharmacological approach for limb girdle muscular dystrophy type 2D patients carrying mutations that impair a-sarcoglycan trafficking. Improves maturation of misfolded proteins.
Research or further manufacturing use only, not for food or drug use.