Mutant INS-gene induced diabetes of youth: proinsulin cysteine residues impose dominant-negative inhibition on wild-type proinsulin transport.

Recently, a syndrome of Mutant INS-gene-induced Diabetes of Youth (MIDY, derived from one of 26 distinct mutations) has been identified as a cause of insulin-deficient diabetes, resulting from expression of a misfolded mutant proinsulin protein in the endoplasmic reticulum (ER) of insulin-producing...

Full description

Bibliographic Details
Main Authors: Ming Liu, Leena Haataja, Jordan Wright, Nalinda P Wickramasinghe, Qing-Xin Hua, Nelson F Phillips, Fabrizio Barbetti, Michael A Weiss, Peter Arvan
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2010-10-01
Series:PLoS ONE
Online Access:http://europepmc.org/articles/PMC2952628?pdf=render