CFTR F508del is degraded by the 26S proteasome

Stable Identifier
R-HSA-8866858
Type
Reaction [uncertain]
Species
Homo sapiens
Compartment
cytosol
ReviewStatus
5/5
Locations in the PathwayBrowser
Expand all
General
SVG | 
PNG
Low
Medium
High
 | PPTX  | SBGN
CFTR F508del is degraded by the 26S proteasome
Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout
After VCP-mediated translocation to the cytosol, misfolded CFTR F508del is degraded by the 26S proteasome (El Khouri et al, 2013).
Literature References
PubMed ID Title Journal Year
24019521 RNF185 is a novel E3 ligase of endoplasmic reticulum-associated degradation (ERAD) that targets cystic fibrosis transmembrane conductance regulator (CFTR)

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

J. Biol. Chem. 2013
Participants
Input
Output
Participates
as an event of
Catalyst Activity

endopeptidase activity of 26S proteasome [cytosol]

Physical Entity
26S proteasome [cytosol] (Homo sapiens)
Activity
endopeptidase activity (GO:0004175)
Normal reaction
misfolded CFTR is degraded by the 26S proteasome (Homo sapiens)
Functional status

Gain of function of ub-CFTR F508del [cytosol]

Normal Entity
Disease Entity
Status
Disease
Name Identifier Synonyms
cystic fibrosis DOID:1485 mucoviscidosis, CF
Authored
Rothfels, K  (2016-04-02)
Rothfels, K  (2016-04-02)
Reviewed
D'Eustachio, P  (2016-04-23)
Created
Rothfels, K  (2016-04-04)
Cite Us!