RNF5 and RNF185 ubiquitinate CFTR F508del

Stable Identifier
R-HSA-8866856
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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RNF5 and RNF185 ubiquitinate CFTR F508del 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
Participates
Catalyst Activity

ubiquitin protein ligase activity of CFTR F508del:ERAD complex [endoplasmic reticulum membrane]

Normal reaction
Functional status

Gain of function of CFTR F508del:ERAD complex [endoplasmic reticulum membrane]

Status
Disease
Name Identifier Synonyms
cystic fibrosis DOID:1485 mucoviscidosis, CF
Authored
Reviewed
Created
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