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

Function: 1) endoplasmic reticulum-associated degradation a) misfolded lumenal glycoproteins, but NOT that of misfolded non-glycoproteins 2) forms channel that allows the retrotranslocation of misfolded glycoproteins into cytosol where they are ubiquitinated & degraded by the proteasome (putative) 3) mediates interaction between VCP & the degradation substrate 4) forms homo- & heterooligomers with DERL2 & DERL3 5) interacts with AMFR, SELS/VIMP, SEL1L, SYVN1, VCP, NGLY1 - does NOT bind to EDEM1 6) up-regulated in the unfolded protein response - via the ERN1-XBP1 pathway Compartment: endoplasmic reticulum membrane Expression: ubiquitous Pathology: 1) export from the ER & subsequent degradation of MHC class 1 heavy chains interaction with cytomegalovirus US11 viral protein 2) degradation of misfolded cytomegalovirus US2 protein

Properties

SIZE: MW = 29 kD entity length = 251 aa COMPARTMENT: endoplasmic reticulum MOTIF: cytoplasmic domain {1-22} transmembrane domain {23-43} exoplasmic loop {44-59} transmembrane domain {60-80} cytoplasmic loop {81-105} transmembrane domain {106-126} exoplasmic loop {127-154} transmembrane domain {155-175} cytoplasmic domain {176-251}

Database Correlations

OMIM 608813 UniProt Q9BUN8 Pfam PF04511

References

UniProt :accession Q9BUN8