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Functional component of endoplasmic reticulum-associated degradation (ERAD) for misfolded lumenal proteins. May act by forming a channel that allows the retrotranslocation of misfolded proteins into the cytosol where they are ubiquitinated and degraded by the proteasome. May mediate the interaction between VCP and the degradation substrate. In case of infection by cytomegaloviruses, it plays a central role in the export from the ER and subsequent degradation of MHC class I heavy chains via its interaction with US11 viral protein, which recognizes and associates with MHC class I heavy chains. Also participates in the degradation process of misfolded cytomegalovirus US2 protein.
Gene Name: | derlin 1 |
Synonyms: | DERL1, DER1, Der1-like protein 1, Derlin-1, DERtrin-1, PRO2577, DER-1, Derlin 1 |
Target Sequences: | NM_024295 NP_077271.1 Q9BUN8 |
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