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
cytosol
ReviewStatus
5/5
Locations in the PathwayBrowser
Expand all
General
SVG | 
PNG
Low
Medium
High
 | PPTX  | SBGN
Hh processing variants are translocated to the cytosol in a VCP-dependent manner
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
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
21357747 Processing and turnover of the Hedgehog protein in the endoplasmic reticulum

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

J. Cell Biol. 2011
23867461 Derlin2 protein facilitates HRD1-mediated retro-translocation of sonic hedgehog at the endoplasmic reticulum

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

J. Biol. Chem. 2013
19002207 One step at a time: endoplasmic reticulum-associated degradation

Vembar, SS, Brodsky, JL

Nat. Rev. Mol. Cell Biol. 2008
Participants
Input
Output
Participates
as an event of
Catalyst Activity

ABC-type transporter activity of ub-SHH processing variants:ERLEC:OS9:SEL1:SYVN1 dimer:DERL2:VCP hexamer [endoplasmic reticulum membrane]

Physical Entity
ub-SHH processing variants:ERLEC:OS9:SEL1:SYVN1 dimer:DERL2:VCP hexamer [endoplasmic reticulum membrane] (Homo sapiens)
Activity
ABC-type transporter activity (GO:0140359)
Functional status

Gain of function of ub-SHH processing variants:ERLEC:OS9:SEL1:SYVN1 dimer:DERL2:VCP hexamer [endoplasmic reticulum membrane]

Disease Entity
Status
Disease
Name Identifier Synonyms
holoprosencephaly DOID:4621 Holoprosencephaly sequence (disorder)
Authored
Rothfels, K  (2014-04-08)
Reviewed
Liu, Y C  (2014-05-16)
Created
Rothfels, K  (2014-04-30)
Cite Us!