CFTR F508del is degraded by the 26S proteasome

Stable Identifier
R-HSA-8866858
Type
Reaction [uncertain]
Species
Homo sapiens
Compartment
cytosol
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CFTR F508del is degraded by the 26S proteasome
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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, Le Pavec, G, Toledano, MB, Delaunay-Moisan, A

J. Biol. Chem. 2013
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Output
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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)
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