C-terminal Hh fragments are recruited to SEL1:SYVN1 at the ER membrane

Stable Identifier
R-HSA-5362441
Type
Reaction [binding]
Species
Homo sapiens
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After binding to OS9/ERLEC1, the Hh C-terminal fragments are recruited to the ER membrane through a lectin-SEL1 interaction. SEL1 is a component of a multiprotein retrotranslocation complex in the ER membrane that also includes the E3 ubiquitin ligase SYVN1 (also known as HRD1; present as a dimer), DERL2 and the hexameric ATPase VCP (Christianson et al, 2008; Hosokawa et al, 2008; Mueller et al, 2008; Chen et al, 2011; Huang et al, 2013; reviewed in Vembar and Brodsky, 2008). Depletion of SEL1, SYVN1, VCP or DERL2 results in the accumulation of the Hh-C in the ER lumen (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
18711132 SEL1L nucleates a protein complex required for dislocation of misfolded glycoproteins

Mueller, B, Klemm, EJ, Spooner, E, Claessen, JH, Ploegh, HL

Proc. Natl. Acad. Sci. U.S.A. 2008
18502753 Human XTP3-B forms an endoplasmic reticulum quality control scaffold with the HRD1-SEL1L ubiquitin ligase complex and BiP

Hosokawa, N, Wada, I, Nagasawa, K, Moriyama, T, Okawa, K, Nagata, K

J. Biol. Chem. 2008
19002207 One step at a time: endoplasmic reticulum-associated degradation

Vembar, SS, Brodsky, JL

Nat. Rev. Mol. Cell Biol. 2008
18264092 OS-9 and GRP94 deliver mutant alpha1-antitrypsin to the Hrd1-SEL1L ubiquitin ligase complex for ERAD

Christianson, JC, Shaler, TA, Tyler, RE, Kopito, RR

Nat. Cell Biol. 2008
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