Hh processing variants are translocated to the cytosol in a VCP-dependent manner

Stable Identifier
R-HSA-5387389
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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Depletion of the ATPase VCP results in the stabilization of processing-defective Hh variants in the ER lumen, supporting the notion that, as is the case for the WT Hh C-terminal fragment, these peptides are also substrates for ERAD (Chen et al, 2011; Huang et al, 2013).

Literature References
PubMed ID Title Journal Year
23867461 Derlin2 protein facilitates HRD1-mediated retro-translocation of sonic hedgehog at the endoplasmic reticulum

Huang, CH, Hsiao, HT, Chu, YR, Ye, Y, Chen, X

J. Biol. Chem. 2013
21357747 Processing and turnover of the Hedgehog protein in the endoplasmic reticulum

Chen, X, Tukachinsky, H, Huang, CH, Jao, C, Chu, YR, Tang, HY, Mueller, B, Schulman, S, Rapoport, TA, Salic, A

J. Cell Biol. 2011
19002207 One step at a time: endoplasmic reticulum-associated degradation

Vembar, SS, Brodsky, JL

Nat. Rev. Mol. Cell Biol. 2008
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
ATPase activity, coupled to transmembrane movement of substances of ub-SHH processing variants:ERLEC:OS9:SEL1:SYVN1 dimer:DERL2:VCP hexamer [endoplasmic reticulum membrane]
Physical Entity
Activity
Disease
Name Identifier Synonyms
holoprosencephaly 4621 Holoprosencephaly sequence (disorder)
Authored
Reviewed
Created