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DERL1 [endoplasmic reticulum membrane]
Stable Identifier
R-HSA-6781898
Type
Protein [EntityWithAccessionedSequence]
Species
Homo sapiens
Compartment
endoplasmic reticulum membrane
Synonyms
Derlin-1, DERL1_HUMAN
Locations in the PathwayBrowser
Expand all
Disease (Homo sapiens)
Disorders of transmembrane transporters (Homo sapiens)
ABC transporter disorders (Homo sapiens)
Defective CFTR causes cystic fibrosis (Homo sapiens)
CFTR F508del binds components of the ERAD machinery for ubiquitination and degradation (Homo sapiens)
CFTR F508del:ERAD complex [endoplasmic reticulum membrane] (Homo sapiens)
ERAD complex [endoplasmic reticulum membrane] (Homo sapiens)
DERL oligomer [endoplasmic reticulum membrane] (Homo sapiens)
DERLs [endoplasmic reticulum membrane] (Homo sapiens)
DERL1 [endoplasmic reticulum membrane] (Homo sapiens)
ERAD complex [endoplasmic reticulum membrane] (Homo sapiens)
DERL oligomer [endoplasmic reticulum membrane] (Homo sapiens)
DERLs [endoplasmic reticulum membrane] (Homo sapiens)
DERL1 [endoplasmic reticulum membrane] (Homo sapiens)
RNF5 and RNF185 ubiquitinate CFTR F508del (Homo sapiens)
CFTR F508del:ERAD complex [endoplasmic reticulum membrane] (Homo sapiens)
ERAD complex [endoplasmic reticulum membrane] (Homo sapiens)
DERL oligomer [endoplasmic reticulum membrane] (Homo sapiens)
DERLs [endoplasmic reticulum membrane] (Homo sapiens)
DERL1 [endoplasmic reticulum membrane] (Homo sapiens)
ub-CFTR F508del:ERAD complex [endoplasmic reticulum membrane] (Homo sapiens)
ERAD complex [endoplasmic reticulum membrane] (Homo sapiens)
DERL oligomer [endoplasmic reticulum membrane] (Homo sapiens)
DERLs [endoplasmic reticulum membrane] (Homo sapiens)
DERL1 [endoplasmic reticulum membrane] (Homo sapiens)
VCP-catalyzed ATP hydrolysis promotes the translocation of CFTR F508del into the cytosol (Homo sapiens)
ERAD complex [endoplasmic reticulum membrane] (Homo sapiens)
DERL oligomer [endoplasmic reticulum membrane] (Homo sapiens)
DERLs [endoplasmic reticulum membrane] (Homo sapiens)
DERL1 [endoplasmic reticulum membrane] (Homo sapiens)
ub-CFTR F508del:ERAD complex [endoplasmic reticulum membrane] (Homo sapiens)
ERAD complex [endoplasmic reticulum membrane] (Homo sapiens)
DERL oligomer [endoplasmic reticulum membrane] (Homo sapiens)
DERLs [endoplasmic reticulum membrane] (Homo sapiens)
DERL1 [endoplasmic reticulum membrane] (Homo sapiens)
Metabolism of proteins (Homo sapiens)
Post-translational protein modification (Homo sapiens)
Asparagine N-linked glycosylation (Homo sapiens)
N-glycan trimming in the ER and Calnexin/Calreticulin cycle (Homo sapiens)
Deglycosylation complex hydrolyses N-glycans from unfolded glycoproteins (Homo sapiens)
Deglycosylation complex [endoplasmic reticulum membrane] (Homo sapiens)
DERL1 [endoplasmic reticulum membrane] (Homo sapiens)
Protein ubiquitination (Homo sapiens)
E3 ubiquitin ligases ubiquitinate target proteins (Homo sapiens)
TMEM129 polyubiquitinates HLA (MHC class I heavy chain) bound to cytomegalovirus US11 (Homo sapiens)
TMEM129:DERL1:VIMP:VCP:UBE2J2 [endoplasmic reticulum membrane] (Homo sapiens)
DERL1 [endoplasmic reticulum membrane] (Homo sapiens)
US11:HLA:TMEM129:DERL1:VIMP:VCP:Ub:UBE2J2 [endoplasmic reticulum membrane] (Homo sapiens)
TMEM129:DERL1:VIMP:VCP:Ub:UBE2J2 [endoplasmic reticulum membrane] (Homo sapiens)
DERL1 [endoplasmic reticulum membrane] (Homo sapiens)
US11:HLA binds DERL1:TMEM129:Ub:UBE2J2,UBE2K:VIMP:VCP (Homo sapiens)
TMEM129:DERL1:VIMP:VCP:Ub:UBE2J2 [endoplasmic reticulum membrane] (Homo sapiens)
DERL1 [endoplasmic reticulum membrane] (Homo sapiens)
US11:HLA:TMEM129:DERL1:VIMP:VCP:Ub:UBE2J2 [endoplasmic reticulum membrane] (Homo sapiens)
TMEM129:DERL1:VIMP:VCP:Ub:UBE2J2 [endoplasmic reticulum membrane] (Homo sapiens)
DERL1 [endoplasmic reticulum membrane] (Homo sapiens)
Transport of small molecules (Homo sapiens)
ABC-family proteins mediated transport (Homo sapiens)
CFTR binds components of the ERAD machinery for ubiquitination and degradation (Homo sapiens)
ERAD complex [endoplasmic reticulum membrane] (Homo sapiens)
DERL oligomer [endoplasmic reticulum membrane] (Homo sapiens)
DERLs [endoplasmic reticulum membrane] (Homo sapiens)
DERL1 [endoplasmic reticulum membrane] (Homo sapiens)
misfolded CFTR:ERAD complex [endoplasmic reticulum membrane] (Homo sapiens)
ERAD complex [endoplasmic reticulum membrane] (Homo sapiens)
DERL oligomer [endoplasmic reticulum membrane] (Homo sapiens)
DERLs [endoplasmic reticulum membrane] (Homo sapiens)
DERL1 [endoplasmic reticulum membrane] (Homo sapiens)
RNF5 and RNF185 ubiquitinate misfolded CFTR (Homo sapiens)
Ub-misfolded nascent CFTR:ERAD complex [endoplasmic reticulum membrane] (Homo sapiens)
ERAD complex [endoplasmic reticulum membrane] (Homo sapiens)
DERL oligomer [endoplasmic reticulum membrane] (Homo sapiens)
DERLs [endoplasmic reticulum membrane] (Homo sapiens)
DERL1 [endoplasmic reticulum membrane] (Homo sapiens)
misfolded CFTR:ERAD complex [endoplasmic reticulum membrane] (Homo sapiens)
ERAD complex [endoplasmic reticulum membrane] (Homo sapiens)
DERL oligomer [endoplasmic reticulum membrane] (Homo sapiens)
DERLs [endoplasmic reticulum membrane] (Homo sapiens)
DERL1 [endoplasmic reticulum membrane] (Homo sapiens)
VCP-catalyzed ATP hydrolysis promotes the translocation of misfolded CFTR into the cytosol (Homo sapiens)
ERAD complex [endoplasmic reticulum membrane] (Homo sapiens)
DERL oligomer [endoplasmic reticulum membrane] (Homo sapiens)
DERLs [endoplasmic reticulum membrane] (Homo sapiens)
DERL1 [endoplasmic reticulum membrane] (Homo sapiens)
Ub-misfolded nascent CFTR:ERAD complex [endoplasmic reticulum membrane] (Homo sapiens)
ERAD complex [endoplasmic reticulum membrane] (Homo sapiens)
DERL oligomer [endoplasmic reticulum membrane] (Homo sapiens)
DERLs [endoplasmic reticulum membrane] (Homo sapiens)
DERL1 [endoplasmic reticulum membrane] (Homo sapiens)
External Reference Information
External Reference
UniProt:Q9BUN8 DERL1
Gene Names
DERL1, DER1, UNQ243/PRO276
Chain
initiator methionine:1, chain:2-251
Reference Genes
BioGPS Gene:79139 DERL1
COSMIC (genes):DERL1 DERL1
CTD Gene:79139 DERL1
dbSNP Gene:79139 DERL1
ENSEMBL:ENSG00000136986 DERL1
HGNC:28454 DERL1
KEGG Gene (Homo sapiens):79139 DERL1
Monarch:79139 DERL1
NCBI Gene:79139 DERL1
OMIM:608813 DERL1
UCSC:Q9BUN8 DERL1
Reference Transcript
RefSeq:NM_001134671.2 DERL1
RefSeq:NM_024295.5 DERL1
Other Identifiers
0005550170
11721635_a_at
11721636_a_at
11721637_s_at
17080693
218172_s_at
219402_s_at
222543_at
3151402
3151403
3151404
3151405
3151406
3151407
3151408
3151410
3151411
3151412
3151413
3151414
3151419
3151422
3151423
3151426
3151427
45326_at
79139
8152628
91552_at
A_23_P216043
A_24_P175347
GE79678
GE82428
GO:0000839
GO:0002020
GO:0003674
GO:0005515
GO:0005575
GO:0005622
GO:0005737
GO:0005768
GO:0005769
GO:0005770
GO:0005783
GO:0005785
GO:0005789
GO:0006457
GO:0006464
GO:0006810
GO:0006950
GO:0006986
GO:0007165
GO:0008150
GO:0009056
GO:0015031
GO:0016020
GO:0016021
GO:0016032
GO:0016567
GO:0019899
GO:0030176
GO:0030433
GO:0030968
GO:0030970
GO:0031398
GO:0031410
GO:0031625
GO:0031648
GO:0032092
GO:0032991
GO:0034620
GO:0036502
GO:0036503
GO:0036513
GO:0038023
GO:0042288
GO:0042802
GO:0043161
GO:0043226
GO:0044403
GO:0045184
GO:0048500
GO:0051117
GO:0051787
GO:0055085
GO:0071712
GO:1990381
Hs.241576.0.S2_3p_at
Hs.323114.1.S1_3p_a_at
ILMN_1695726
ILMN_2209748
PH_hs_0012961
TC08001575.hg
g8924181_3p_a_at
Participant Of
hasComponent
Deglycosylation complex [endoplasmic reticulum membrane]
TMEM129:DERL1:VIMP:VCP:Ub:UBE2J2 [endoplasmic reticulum membrane]
TMEM129:DERL1:VIMP:VCP:UBE2J2 [endoplasmic reticulum membrane]
hasMember
DERLs [endoplasmic reticulum membrane]
Inferred To
Derl1 [endoplasmic reticulum membrane] (Mus musculus)
Derl1 [endoplasmic reticulum membrane] (Rattus norvegicus)
derl1 [endoplasmic reticulum membrane] (Danio rerio)
derl1 [endoplasmic reticulum membrane] (Xenopus tropicalis)
cup-2 [endoplasmic reticulum membrane] (Caenorhabditis elegans)
DERL1 [endoplasmic reticulum membrane] (Canis familiaris)
DERL1 [endoplasmic reticulum membrane] (Gallus gallus)
DERL1 [endoplasmic reticulum membrane] (Sus scrofa)
Der-1 [endoplasmic reticulum membrane] (Drosophila melanogaster)
Cross References
RefSeq
NP_077271.1
,
NP_001128143.1
OpenTargets
ENSG00000136986
GeneCards
Q9BUN8
HPA
ENSG00000136986-DERL1
Ensembl
ENST00000405944
,
ENST00000259512
,
ENSP00000259512
,
ENSG00000136986
,
ENSP00000384289
PRO
Q9BUN8
PDB
5GLF
Interactors (9)
Accession
#Entities
Entities
Confidence Score
Evidence (IntAct)
UniProt:O15552 FFAR2
1
FFAR2 [plasma membrane]
(R-HSA-443944)
0.556
3
UniProt:Q3KNW5 SLC10A6
1
SLC10A6 [plasma membrane]
(R-HSA-433091)
0.556
3
UniProt:Q14973 SLC10A1
1
SLC10A1 [plasma membrane]
(R-HSA-194116)
0.556
3
UniProt:Q05329 GAD2
2
PXLP-K396-GAD2 [plasma membrane]
(R-HSA-936509)
PXLP-K396-GAD2 [clathrin-sculpted gamma-aminobutyric acid transport vesicle membrane]
(R-HSA-888582)
0.556
3
UniProt:Q8TB40 ABHD4
1
ABHD4 [endoplasmic reticulum membrane]
(R-HSA-5694582)
0.556
3
UniProt:Q8IWU4 SLC30A8
1
SLC30A8 [secretory granule membrane]
(R-HSA-437106)
0.556
3
UniProt:P55072 VCP
8
VCP [endoplasmic reticulum membrane]
(R-HSA-8943105)
Me3K315-VCP [cytosol]
(R-HSA-8932209)
VCP [nucleoplasm]
(R-HSA-5643745)
VCP [azurophil granule lumen]
(R-HSA-6800438)
VCP [ficolin-1-rich granule lumen]
(R-HSA-6800425)
VCP [extracellular region]
(R-HSA-6806191)
VCP [secretory granule lumen]
(R-HSA-6801039)
VCP [cytosol]
(R-HSA-1181251)
0.527
2
UniProt:P51572 BCAP31
5
BCAP31(165-237) [endoplasmic reticulum membrane]
(R-HSA-351909)
BCAP31(2-245) [endoplasmic reticulum membrane]
(R-HSA-201587)
BCAP31(2-164) [endoplasmic reticulum membrane]
(R-HSA-351826)
BCAP31(238-246) [cytosol]
(R-HSA-351912)
BCAP31 [integral component of lumenal side of endoplasmic reticulum membrane]
(R-HSA-985503)
0.499
3
UniProt:P13569 CFTR
17
PolyUb-CFTR [plasma membrane]
(R-HSA-8867595)
PolyUb-CFTR [clathrin-coated endocytic vesicle membrane]
(R-HSA-8869104)
CFTR [lysosomal membrane]
(R-HSA-5627279)
CFTR [Golgi-associated vesicle membrane]
(R-HSA-5627079)
CFTR [endosome membrane]
(R-HSA-6782965)
PolyUb-CFTR [endosome membrane]
(R-HSA-6782976)
CFTR F508del [endoplasmic reticulum membrane]
(R-HSA-8866834)
ub-CFTR F508del [cytosol]
(R-HSA-8866838)
ub-CFTR F508del [endoplasmic reticulum membrane]
(R-HSA-8866842)
CFTR G542* [plasma membrane]
(R-HSA-5678986)
CFTR G551D [plasma membrane]
(R-HSA-5678977)
CFTR W1282* [plasma membrane]
(R-HSA-5678976)
CFTR F508del [plasma membrane]
(R-HSA-5678978)
CFTR N1303K [plasma membrane]
(R-HSA-5678983)
CFTR [plasma membrane]
(R-HSA-383188)
CFTR [endoplasmic reticulum membrane]
(R-HSA-8866837)
misfolded CFTR [endoplasmic reticulum membrane]
(R-HSA-8868730)
0.499
2
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