VCP-catalyzed ATP hydrolysis promotes the translocation of CFTR F508del into the cytosol

Stable Identifier
R-HSA-8866854
Type
Reaction [dissociation]
Species
Homo sapiens
Compartment
endoplasmic reticulum membrane
ReviewStatus
5/5
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VCP-catalyzed ATP hydrolysis promotes the translocation of CFTR F508del into the cytosol
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Retrotranslocation of the misfolded CFTR F508del likely depends on the ERAD ATPase VCP (reviewed in Vembar and Brodsky, 2008; Pranke and Sermet-Gaudelus, 2014).
Literature References
PubMed ID Title Journal Year
24685677 Biosynthesis of cystic fibrosis transmembrane conductance regulator

Pranke, IM, Sermet-Gaudelus, I

Int. J. Biochem. Cell Biol. 2014
19002207 One step at a time: endoplasmic reticulum-associated degradation

Vembar, SS, Brodsky, JL

Nat. Rev. Mol. Cell Biol. 2008
Participants
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Output
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as an event of
Normal reaction
VCP-catalyzed ATP hydrolysis promotes the translocation of misfolded CFTR into the cytosol (Homo sapiens)
Functional status

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

Normal Entity
Disease Entity
Status
Disease
Name Identifier Synonyms
cystic fibrosis DOID:1485 mucoviscidosis, CF
Cross References
Mondo
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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