ID:DERL1_HUMAN DESCRIPTION: RecName: Full=Derlin-1; AltName: Full=Degradation in endoplasmic reticulum protein 1; Short=DERtrin-1; AltName: Full=Der1-like protein 1; 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. INTERACTION: P51572:BCAP31; NbExp=3; IntAct=EBI-398977, EBI-77683; P13569:CFTR; NbExp=2; IntAct=EBI-398977, EBI-349854; SUBCELLULAR LOCATION: Endoplasmic reticulum membrane; Multi-pass membrane protein. TISSUE SPECIFICITY: Ubiquitous. INDUCTION: Up-regulated in response to endoplasmic reticulum stress via the ERN1-XBP1 pathway of the unfolded protein response (UPR) (By similarity). SIMILARITY: Belongs to the derlin family.
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
ModBase Predicted Comparative 3D Structure on Q9BUN8
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.
Biological Process: GO:0006457 protein folding GO:0006986 response to unfolded protein GO:0015031 protein transport GO:0016032 viral process GO:0016567 protein ubiquitination GO:0030433 ER-associated ubiquitin-dependent protein catabolic process GO:0030968 endoplasmic reticulum unfolded protein response GO:0030970 retrograde protein transport, ER to cytosol GO:0031398 positive regulation of protein ubiquitination GO:0031648 protein destabilization GO:0032092 positive regulation of protein binding GO:0034620 cellular response to unfolded protein GO:0036503 ERAD pathway GO:0043161 proteasome-mediated ubiquitin-dependent protein catabolic process GO:0045184 establishment of protein localization GO:0051260 protein homooligomerization GO:0055085 transmembrane transport GO:0071712 ER-associated misfolded protein catabolic process
Cellular Component: GO:0005769 early endosome GO:0005770 late endosome GO:0005783 endoplasmic reticulum GO:0005789 endoplasmic reticulum membrane GO:0016020 membrane GO:0016021 integral component of membrane GO:0030176 integral component of endoplasmic reticulum membrane GO:0036502 Derlin-1-VIMP complex GO:0036513 Derlin-1 retrotranslocation complex GO:0005785 signal recognition particle receptor complex GO:0034098 VCP-NPL4-UFD1 AAA ATPase complex GO:0048500 signal recognition particle
Protein Q9BUN8 (Reactome details) participates in the following event(s):
R-HSA-8943083 US11:HLA binds DERL1:TMEM129:Ub:UBE2J2,UBE2K:VIMP:VCP R-HSA-8850594 Deglycosylation complex hydrolyses N-glycans from unfolded glycoproteins R-HSA-8866551 CFTR binds components of the ERAD machinery for ubiquitination and degradation R-HSA-8866857 CFTR F508del binds components of the ERAD machinery for ubiquitination and degradation R-HSA-8866542 VCP-catalyzed ATP hydrolysis promotes the translocation of misfolded CFTR into the cytosol R-HSA-8866854 VCP-catalyzed ATP hydrolysis promotes the translocation of CFTR F508del into the cytosol R-HSA-8866546 RNF5 and RNF185 ubiquitinate misfolded CFTR R-HSA-8866856 RNF5 and RNF185 ubiquitinate CFTR F508del R-HSA-8866654 E3 ubiquitin ligases ubiquitinate target proteins R-HSA-532668 N-glycan trimming in the ER and Calnexin/Calreticulin cycle R-HSA-8852135 Protein ubiquitination R-HSA-446203 Asparagine N-linked glycosylation R-HSA-597592 Post-translational protein modification R-HSA-382556 ABC-family proteins mediated transport R-HSA-5678895 Defective CFTR causes cystic fibrosis R-HSA-392499 Metabolism of proteins R-HSA-382551 Transport of small molecules R-HSA-5619084 ABC transporter disorders R-HSA-5619115 Disorders of transmembrane transporters R-HSA-1643685 Disease
US Server
Sequence and Links to Tools and Databases 
Common Gene Haplotype Alleles 
