background:
Degradation in endoplasmic reticulum proteins, also designated Derlins or DERtrins, are crucial for the degradation of misfolded endoplasmic reticulum (ER) luminal proteins. Derlin proteins are multi-pass membrane proteins localizing to the ER. Derlins are involved in transferring misfolded proteins from the ER to the cytosol, where the misfolded proteins are destroyed in an ubiquitin-dependent manner by the proteasome. In the case of cytomegalovirus infection, Derlin-1, as opposed to Derlins-2 and -3, is involved in the export of MHC class I heavy chains from the ER via its interaction with the viral protein US11. Derlins may also be important for cell proliferation.
Function:
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.
Subunit:
Forms homo- and heterooligomers with DERL2 and DERL3; binding to DERL3 is poorer than that between DERL2 and DERL3. Interacts with AMFR, VIMP/SELS, SEL1L, SYVN1 and VCP, as well as with SEL1L-SYVN1 and VCP-VIMP protein complexes; this interaction is weaker than that observed between DERL2 and these complexes. Interacts with the cytomegalovirus US11 protein. Interacts with NGLY1 and YOD1. Does not bind to EDEM1. Interacts with RNF103.
Subcellular Location:
Endoplasmic reticulum membrane; Multi-pass membrane protein.
Tissue Specificity:
Ubiquitous.
Similarity:
Belongs to the derlin family.
Database links:
Entrez Gene: 420350 Chicken
Entrez Gene: 404121 Cow
Entrez Gene: 475086 Dog
Entrez Gene: 79139 Human
Entrez Gene: 67819 Mouse
Entrez Gene: 10065362 Pig
Entrez Gene: 362912 Rat
Omim: 608813 Human
SwissProt: Q71SS4 Cow
SwissProt: Q9BUN8 Human
SwissProt: Q99J56 Mouse
Unigene: 241576 Human
Unigene: 289387 Mouse
Unigene: 110990 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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