derlin-1 (degradation in endoplasmic reticulum protein 1, Der1-like protein 1, DERtrin-1, DERL1, DER1, UNQ243/PRO276)

Function

• endoplasmic reticulum-associated degradation
  • misfolded lumenal glycoproteins, but NOT that of misfolded non-glycoproteins
• forms channel that allows the retrotranslocation of misfolded glycoproteins into cytosol where they are ubiquitinated & degraded by the proteasome (putative)
• mediates interaction between VCP & the degradation substrate
• forms homo- & heterooligomers with DERL2 & DERL3
• interacts with AMFR, SELS/VIMP, SEL1L, SYVN1, VCP, NGLY1
  • does NOT bind to EDEM1
• up-regulated in the unfolded protein response
  • via the ERN1-XBP1 pathway

Compartment

endoplasmic reticulum membrane

Expression

ubiquitous

Pathology

• export from the ER & subsequent degradation of MHC class 1 heavy chains interaction with cytomegalovirus US11 viral protein
• degradation of misfolded cytomegalovirus US2 protein

More general terms

• derlin

Database

• OMIM: https://mirror.omim.org/entry/608813
• UniProt: http://www.uniprot.org/uniprot/Q9BUN8.html