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.
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
PUBLICATION RECORD
- Publication year
2013
- Venue
Molecular Biology of the Cell
- Publication date
2013-01-15
- Fields of study
Biology, Medicine
- Identifiers
- External record
- Source metadata
Semantic Scholar, PubMed
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