Article
Chaperone-Driven Degradation of a Misfolded Proinsulin Mutant in Parallel With Restoration of Wild-Type Insulin Secretion.
Diabetes - 1 Mar 2017
Cunningham Corey N, He Kaiyu, Arunagiri Anoop, Paton Adrienne W, Paton James C, Arvan Peter, Tsai Billy
Abstract excerpt
In heterozygous patients with a diabetic syndrome called mutant INS gene-induced diabetes of youth (MIDY), there is decreased insulin secretion when mutant proinsulin expression prevents wild-type (WT) proinsulin from exiting the endoplasmic reticulum (ER), which is essential for insulin production. Our previous results revealed that mutant Akita proinsulin is triaged by ER-associated degradation (ERAD). We now...
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