Small heat shock proteins target mutant cystic fibrosis transmembrane conductance regulator for degradation via a small ubiquitin-like modifier–dependent pathway

A. Ahner,Xiaoyan Gong,B. Schmidt,K. Peters,W. Rabeh,P. Thibodeau,G. Lukács,R. Frizzell

Published 2013 in Molecular Biology of the Cell

ABSTRACT

Selective degradation of the mutant protein responsible for most cystic fibrosis, F508del cystic fibrosis transmembrane conductance regulator (CFTR), is initiated by Hsp27, which associates with the small ubiquitin-like modifier (SUMO) E2, Ubc9. They modify F508del with SUMO-2/3, directing F508del to a SUMO-targeted ubiquitin ligase, RNF4. This work implicates SUMO and RNF4 in quality control of a cytosolic transmembrane protein.

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