Misfolded proinsulin affects bystander proinsulin in neonatal diabetes

I Hodish, M Liu, G Rajpal, D Larkin, RW Holz… - Journal of biological …, 2010 - ASBMB
It has previously been shown that misfolded mutant Akita proinsulin in the endoplasmic
reticulum engages directly in protein complexes either with nonmutant proinsulin or with"
hProCpepGFP"(human proinsulin bearing emerald-GFP within the C-peptide), impairing the
trafficking of these" bystander" proinsulin molecules (Liu, M., Hodish, I., Rhodes, CJ, and
Arvan, P.(2007) Proc. Natl. Acad. Sci. USA 104, 15841–15846). Herein, we generated
transgenic mice, which, in addition to expressing endogenous proinsulin, exhibit β-cell …