Hh processing variants are recruited to SEL1:SYVN at the ER membrane

Stable Identifier
R-HSA-5387386
Type
Reaction [binding]
Species
Homo sapiens
Compartment
endoplasmic reticulum membrane
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Hh processing variants are recruited to SEL1:SYVN at the ER membrane
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As is the case for the WT Hh C-terminal fragment, siRNA depletion of SEL1 and SYVN1 inhibits the degradation of processing-defective Hh mutants, suggesting these versions of Hh are also targets for ERAD-mediated degradation (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
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Output
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as an event of
Functional status

Gain of function of SHH processing variants:OS9/ERLEC1 [endoplasmic reticulum lumen]

Disease Entity
Status
Disease
Name Identifier Synonyms
holoprosencephaly DOID:4621 Holoprosencephaly sequence (disorder)
Authored
Rothfels, K  (2014-04-21)
Reviewed
Liu, Y C  (2014-05-16)
Created
Rothfels, K  (2014-04-30)
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