RNF5 and RNF185 ubiquitinate misfolded CFTR

Stable Identifier
R-HSA-8866546
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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RNF5 and RNF185 ubiquitinate misfolded CFTR as part of the retrotranslocon that targets the receptor for degradation through the ERAD pathway. Both depletion of the E3 ligases by siRNA and expression of a catalytically inactive form of the enzyme strongly inhibits CFTR degradation (El Khouri et al, 2013; Younger et al, 2006).

Literature References
PubMed ID Title Journal Year
16901789 Sequential quality-control checkpoints triage misfolded cystic fibrosis transmembrane conductance regulator

Younger, JM, Chen, L, Ren, HY, Rosser, MF, Turnbull, EL, Fan, CY, Patterson, C, Cyr, DM

Cell 2006
24019521 RNF185 is a novel E3 ligase of endoplasmic reticulum-associated degradation (ERAD) that targets cystic fibrosis transmembrane conductance regulator (CFTR)

El Khouri, E, Le Pavec, G, Toledano, MB, Delaunay-Moisan, A

J. Biol. Chem. 2013
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
ubiquitin protein ligase activity of misfolded CFTR:ERAD complex [endoplasmic reticulum membrane]
Physical Entity
Activity
Orthologous Events
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Reviewed
Created