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DERL1
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):hsa: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
16562
17080693
218172_s_at
219402_s_at
222543_at
37269
45326_at
5GLF
79139
8152628
91552_at
A0A024R9G3
A_23_P216043
A_24_P175347
AAH02457
AAQ89177
AC104316
AK124086
AY358818
B4E1G1
BAG54003
BC002457
CAB037269
CCDS47915
CCDS6337
CH471060
DERL1
DERL1-201
DERL1-202
EAW92013
EAW92016
ENSG00000136986
ENSP00000259512
ENSP00000384289
ENST00000259512
ENST00000405944
EntrezGene:79139
g8924181_3p_a_at
GE79678
GE82428
GO:0002020
GO:0003674
GO:0004252
GO:0004872
GO:0005515
GO:0005575
GO:0005622
GO:0005623
GO:0005737
GO:0005768
GO:0005769
GO:0005770
GO:0005783
GO:0005785
GO:0005789
GO:0006457
GO:0006461
GO:0006464
GO:0006508
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:0022607
GO:0030176
GO:0030433
GO:0030968
GO:0030970
GO:0031398
GO:0031410
GO:0031625
GO:0031648
GO:0032092
GO:0034098
GO:0034620
GO:0036502
GO:0036503
GO:0036513
GO:0042288
GO:0043226
GO:0043234
GO:0043657
GO:0044403
GO:0045184
GO:0048500
GO:0051117
GO:0051260
GO:0055085
GO:0065003
GO:0071712
GO:1990381
HGNC:28454
HPA016562
Hs.241576
Hs.241576.0.S2_3p_at
Hs.323114.1.S1_3p_a_at
ILMN_1695726
ILMN_2209748
IPR007599
MIM:608813
NM_001134671
NM_024295
NP_001128143
NP_077271
PF04511
PH_hs_0012961
TC08001575.hg
uc003ypl.3
uc003ypm.4
UPI0000048EAA
UPI0000E21C3A
Participant Of
hasMember
DERLs [endoplasmic reticulum membrane]
hasComponent
Deglycosylation complex [endoplasmic reticulum membrane]
TMEM129:DERL1:VIMP:VCP:Ub:UBE2J2 [endoplasmic reticulum membrane]
TMEM129:DERL1:VIMP:VCP:UBE2J2 [endoplasmic reticulum membrane]
Inferred To
Homologues of DERL1 [endoplasmic reticulum membrane] (Danio rerio)
Homologues of DERL1 [endoplasmic reticulum membrane] (Schizosaccharomyces pombe)
Homologues of DERL1 [endoplasmic reticulum membrane] (Taeniopygia guttata)
DERL1 [endoplasmic reticulum membrane] (Arabidopsis thaliana)
DERL1 [endoplasmic reticulum membrane] (Bos taurus)
DERL1 [endoplasmic reticulum membrane] (Caenorhabditis elegans)
DERL1 [endoplasmic reticulum membrane] (Canis familiaris)
DERL1 [endoplasmic reticulum membrane] (Danio rerio)
DERL1 [endoplasmic reticulum membrane] (Dictyostelium discoideum)
DERL1 [endoplasmic reticulum membrane] (Drosophila melanogaster)
DERL1 [endoplasmic reticulum membrane] (Gallus gallus)
DERL1 [endoplasmic reticulum membrane] (Mus musculus)
DERL1 [endoplasmic reticulum membrane] (Oryza sativa)
DERL1 [endoplasmic reticulum membrane] (Rattus norvegicus)
DERL1 [endoplasmic reticulum membrane] (Schizosaccharomyces pombe)
DERL1 [endoplasmic reticulum membrane] (Sus scrofa)
DERL1 [endoplasmic reticulum membrane] (Taeniopygia guttata)
DERL1 [endoplasmic reticulum membrane] (Xenopus tropicalis)
Cross References
RefSeq
NP_077271.1
,
NP_001128143.1
Brenda
3.4.21.105
GeneCards
Q9BUN8
DOCK Blaster
5GLF
PRO
Q9BUN8
PDB
5GLF
Interactors (2)
Accession
#Entities
Entities
Confidence Score
Evidence (IntAct)
UniProt:P13569 CFTR
17
PolyUb-CFTR [plasma membrane]
(R-HSA-8867595)
PolyUb-CFTR [clathrin-coated 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)
misfolded CFTR [endoplasmic reticulum membrane]
(R-HSA-8868730)
CFTR [plasma membrane]
(R-HSA-383188)
CFTR [endoplasmic reticulum membrane]
(R-HSA-8866837)
0.499
2
UniProt:P51572 BCAP31
5
BCAP31(238-246) [cytosol]
(R-HSA-351912)
BCAP31 [integral component of lumenal side of endoplasmic reticulum membrane]
(R-HSA-985503)
BCAP31(2-164) [endoplasmic reticulum membrane]
(R-HSA-351826)
BCAP31(165-237) [endoplasmic reticulum membrane]
(R-HSA-351909)
BCAP31(2-245) [endoplasmic reticulum membrane]
(R-HSA-201587)
0.499
3
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