Gene:
Der1-like domain family, member 1
Gene ID:
199214
Symbol:
DERL1
Species:
1000
Description:
Specifically required for the degradation process of misfolded endoplasmic reticulum (ER) luminal proteins. Participates in the transfer of misfolded proteins from the ER to the cytosol, where they are destroyed by the proteasome in a ubiquitin-dependent manner. May act by forming a channel that allows the retro-translocation of misfolded proteins into the cytosol and transfer them to the ATPase VCP complex, which translocates and ubiquitinates misfolded proteins. In case of infection by the cytomegalovirus, it plays a central role in the export from the ER and subsequent degredation 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.