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

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

Stable Identifier

R-HSA-5362441

Type

Reaction [binding]

Species

Homo sapiens

Compartment

endoplasmic reticulum membrane

ReviewStatus

5/5

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

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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
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
18711132	SEL1L nucleates a protein complex required for dislocation of misfolded glycoproteins Ploegh, HL, Spooner, E, Klemm, EJ, Claessen, JH, Mueller, B	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, Nagata, K, Wada, I, Okawa, K, Nagasawa, K, Moriyama, T	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 Tyler, RE, Christianson, JC, Kopito, RR, Shaler, TA	Nat. Cell Biol.	2008