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Basic Information Integrated Annotations
Tag Content
iUUCD ID IUUC-Hsa-046028
UUCD1 version UUC-HoS-00107
Ensembl Protein ID ENSP00000265038.5
UniProt Accession Q13216; B2RB64; Q6FHX5; Q96GB9; ERCC8_HUMAN
Genbank Protein ID AAA82605.1; CAG38800.1; AAV38824.1; AAO21128.1; BAG37111.1; EAW55004.1; AAH09793.1
Protein Name DNA excision repair protein ERCC-8; Cockayne syndrome WD repeat protein CSA
Genbank Nucleotide ID U28413; CR536563; BT020021; AY213194; AK314511; CH471123; BC009793
Gene Name ERCC8; CKN1; CSA
Ensembl Information
Ensembl Gene ID Ensembl Transcript ID Ensembl Protein ID
ENSG00000049167.13 ENST00000265038.9 ENSP00000265038.5
Annotation
Cancer Mutation
TCGAICGCCOSMICCGAP
IntOGen
Single Nucleotide Polymorphisms (SNP)
dbSNP
mRNA Expression
GEOArrayExpressTCGAICGC
COSMIC
DNA & RNA Element
AREsitecircBasecircRNADbCircNet
miRTarBasemicroRNATRANSFACmiRWalk
RepTarSomamiRmiRcodeRAID2
Protein-protein Interaction
IIDiRefIndexPINAHINT
MenthaInWeb_IM
Protein 3D Structure
PDBMMDB
Disease-associated Information
ClinVarGWASdbOMIM
Post-translational Modifications (PTMs)
CPLMdbPAFPhosphositePlusdbPTM
HPRDUniProtBioGRIDmUbiSiDa
DNA Methylation
TCGAICGCCOSMIC
Protein Expression/Proteomics
THPAGPMDB
Status Reviewed
Details
Family Domain References (PMIDs)
E3 adaptor/Cullin RING/DCX/DWD WD_repeat 18794354; 18936169
Classification
Family E-value Score Start End
E3 adaptor/Cullin RING/DCX/DWD 1.20e-29 102.7 184 218
UBD/Beta-Prp 3.20e-40 139.8 161 361
Active Site
Position(s) Description Evidence
N/A N/A N/A
Domain Profile

   E3 adaptor/Cullin RING/DCX/DWD
   S: 1    gHtdsvtclaf...sdtllvsGsdDgtikvWDlrt 32
           gH  ++ ++++    d +l+++s D  +k+WD+r+
   Q: 184  GHRQEILAVSWsprYDYILATASADSRVKLWDVRR 218
           8**********96543678*************986 PP
   

   UBD/Beta-Prp
   S: 69   tgseDrtikvwdlstgkcvhtlkghedsvlslsfsp.sgdklvsGshdrtirvwdletgq.clee..............tlsghdgvvncvv 144
            g +   +++ dl++g+c h+l+gh +++l++s+sp +++ l+++s+d+ +++wd++  + cl +               ++ h+g vn+++
   Q: 161  VGTRGPKVQLCDLKSGSCSHILQGHRQEILAVSWSPrYDYILATASADSRVKLWDVRRASgCLITldqhngkksqavesANTAHNGKVNGLC 252
           555666677889************************888999**************987636666*************************** PP
   S: 145  vhpdgnllvsGslDrtvrlWdlktgkllrtl....tgghtdsvyslsfdsngkelvsgsldgtvklwdlqtgecvrtlnghtdnvksvrfsp 232
           + +dg  l + ++D+ +rlW++++g+           + ++  + +     + e+v + + +t+ ++ + +ge +  l+gh ++v +  f +
   Q: 253  FTSDGLHLLTVGTDNRMRLWNSSNGENTLVNygkvCNNSKKGLKFTVSCGCSSEFVFVPYGSTIAVYTVYSGEQITMLKGHYKTVDCCVFQS 344
           *****99*************996654333334444223444444444455559*************************************** PP
   S: 233  nderlvsgssDgsiriW 249
           n + l+sgs D  i  W
   Q: 345  NFQELYSGSRDCNILAW 361
           ***************99 PP
   
Organism Homo sapiens
Functional Description
(View)

Functional Description


     Substrate-recognition component of the CSA complex, a DCX (DDB1-CUL4-X-box) E3 ubiquitin-protein ligase complex, involved in transcription-coupled nucleotide excision repair. The CSA complex (DCX(ERCC8) complex) promotes the ubiquitination and subsequent proteasomal degradation of ERCC6 in a UV-dependent manner; ERCC6 degradation is essential for the recovery of RNA synthesis after transcription-coupled repair. It is required for the recruitment of XAB2, HMGN1 and TCEA1/TFIIS to a transcription-coupled repair complex which removes RNA polymerase II-blocking lesions from the transcribed strand of active genes.
 Substrate-recognition component of the CSA complex, a DCX (DDB1-CUL4-X-box) E3 ubiquitin-protein ligase complex, involved in transcription-coupled nucleotide excision repair. The CSA complex (DCX(ERCC8) complex) promotes the ubiquitination and subsequent proteasomal degradation of ERCC6 in a UV-dependent manner; ERCC6 degradation is essential for the recovery of RNA synthesis after transcription-coupled repair. It is required for the recruitment of XAB2, HMGN1 and TCEA1/TFIIS to a transcription-coupled repair complex which removes RNA polymerase II-blocking lesions from the transcribed strand of active genes.
Protein Sequence
(Fasta)
MLGFLSARQT GLEDPLRLRR AESTRRVLGL ELNKDRDVER IHGGGINTLD IEPVEGRYML 60
SGGSDGVIVL YDLENSSRQS YYTCKAVCSI GRDHPDVHRY SVETVQWYPH DTGMFTSSSF 120
It may take some time, please wait.

Protein Fasta Sequence


>IUUC-Hsa-046028|E3,WD_repeat;UBD,Beta-Prp|Homo sapiens
Please wait for a moment...
Nucleotide Sequence
(Fasta)
TCCAGTCCCG CCCCTGAGCT CTGTCATGGC GACGTCCAGT GCTCCAGCCG GTGTGAGGAC 60
ACGATATGCT GGGGTTTTTG TCCGCACGCC AAACGGGTTT GGAGGACCCT CTTCGCCTTC 120
It may take some time, please wait.

Nucleotide Fasta Sequence


>IUUC-Hsa-046028|E3,WD_repeat;UBD,Beta-Prp|Homo sapiens
Please wait for a moment...
Sequence Source Ensembl
Keyword

KW-0002--3D-structure
KW-0025--Alternative splicing
KW-0172--Cockayne syndrome
KW-0181--Complete proteome
KW-0209--Deafness
KW-0225--Disease mutation
KW-0227--DNA damage
KW-0234--DNA repair
KW-0242--Dwarfism
KW-0539--Nucleus
KW-0597--Phosphoprotein
KW-0621--Polymorphism
KW-1185--Reference proteome
KW-0677--Repeat
KW-0833--Ubl conjugation pathway
KW-0853--WD repeat
Interpro

IPR020472--G-protein_beta_WD-40_rep
IPR015943--WD40/YVTN_repeat-like_dom
IPR001680--WD40_repeat
IPR019775--WD40_repeat_CS
IPR017986--WD40_repeat_dom
PROSITE

PS00678--WD_REPEATS_1
PS50082--WD_REPEATS_2
PS50294--WD_REPEATS_REGION
Pfam

PF00400--WD40
PRINTS

PR00320--GPROTEINBRPT
SMART

SM00320--WD40
Gene Ontology

GO:0031464--C:Cul4A-RING E3 ubiquitin ligase complex
GO:0016363--C:nuclear matrix
GO:0005654--C:nucleoplasm
GO:0000109--C:nucleotide-excision repair complex
GO:0005634--C:nucleus
GO:0043234--C:protein complex
GO:0032403--F:protein complex binding
GO:0006974--P:cellular response to DNA damage stimulus
GO:0006289--P:nucleotide-excision repair
GO:0045739--P:positive regulation of DNA repair
GO:0043161--P:proteasome-mediated ubiquitin-dependent protein catabolic process
GO:0051865--P:protein autoubiquitination
GO:0000209--P:protein polyubiquitination
GO:0006979--P:response to oxidative stress
GO:0009411--P:response to UV
GO:0010165--P:response to X-ray
GO:0006283--P:transcription-coupled nucleotide-excision repair
KEGG hsa:1161
Orthology
iUUCD ID Species Identity E-value Score
IUUC-Ata-001170 Aegilops tauschii 30.09 8.00e-37 149.00
IUUC-Aml-002201 Ailuropoda melanoleuca 93.94 0.00e+00 787.00
IUUC-Atr-002784 Amborella trichopoda 37.18 6.00e-41 161.00
IUUC-Apl-003202 Anas platyrhynchos 81.32 0.00e+00 658.00
IUUC-Aca-004708 Anolis carolinensis 80.46 1.00e-176 612.00
IUUC-Aly-006144 Arabidopsis lyrata 30.15 5.00e-44 172.00
IUUC-Ath-006673 Arabidopsis thaliana 39.71 5.00e-45 175.00
IUUC-Acl-008195 Aspergillus clavatus 38.29 7.00e-43 168.00
IUUC-Afl-008406 Aspergillus flavus 36.36 7.00e-39 154.00
IUUC-Afu-008759 Aspergillus fumigatus 36.06 6.00e-40 158.00
IUUC-Ani-009380 Aspergillus nidulans 35.10 7.00e-41 161.00
IUUC-Ang-009820 Aspergillus niger 36.76 4.00e-39 155.00
IUUC-Aor-010092 Aspergillus oryzae 36.67 1.00e-43 170.00
IUUC-Ate-010491 Aspergillus terreus 36.30 2.00e-44 173.00
IUUC-Bta-012825 Bos taurus 93.95 0.00e+00 780.00
IUUC-Bci-013856 Botrytis cinerea 31.58 3.00e-52 199.00
IUUC-Bdi-014899 Brachypodium distachyon 32.84 5.00e-64 238.00
IUUC-Bol-015337 Brassica oleracea 37.93 1.00e-44 174.00
IUUC-Bra-018089 Brassica rapa 37.93 1.00e-44 174.00
IUUC-Cja-018725 Callithrix jacchus 96.97 0.00e+00 805.00
IUUC-Cfa-021162 Canis familiaris 94.44 0.00e+00 789.00
IUUC-Cpo-022190 Cavia porcellus 94.44 0.00e+00 792.00
IUUC-Cre-022960 Chlamydomonas reinhardtii 37.14 4.00e-40 159.00
IUUC-Csa-023610 Chlorocebus sabaeus 100.00 0.00e+00 826.00
IUUC-Cho-024689 Choloepus hoffmanni 92.70 0.00e+00 721.00
IUUC-Cin-025324 Ciona intestinalis 40.16 1.00e-80 293.00
IUUC-Csv-026059 Ciona savignyi 38.37 3.00e-73 269.00
IUUC-Cgl-026384 Colletotrichum gloeosporioides 33.23 1.00e-38 154.00
IUUC-Cme-027182 Cyanidioschyzon merolae 28.66 5.00e-13 69.70
IUUC-Dre-028696 Danio rerio 72.50 1.00e-176 612.00
IUUC-Dno-029102 Dasypus novemcinctus 94.44 0.00e+00 792.00
IUUC-Dor-030361 Dipodomys ordii 77.02 3.00e-179 621.00
IUUC-Dse-031167 Dothistroma septosporum 29.46 2.00e-44 173.00
IUUC-Ete-032856 Echinops telfairi 75.51 6.00e-178 616.00
IUUC-Eca-033611 Equus caballus 95.56 0.00e+00 683.00
IUUC-Eeu-034583 Erinaceus europaeus 71.72 4.00e-162 564.00
IUUC-Fca-036481 Felis catus 94.44 0.00e+00 791.00
IUUC-Fal-037327 Ficedula albicollis 77.94 3.00e-165 573.00
IUUC-Fox-037684 Fusarium oxysporum 30.52 1.00e-43 171.00
IUUC-Fso-038306 Fusarium solani 32.55 5.00e-54 205.00
IUUC-Gmo-039043 Gadus morhua 67.85 4.00e-163 567.00
IUUC-Ggr-039783 Gaeumannomyces graminis 33.67 9.00e-38 151.00
IUUC-Gga-041085 Gallus gallus 85.06 0.00e+00 632.00
IUUC-Gac-042328 Gasterosteus aculeatus 67.08 1.00e-158 552.00
IUUC-Gma-042916 Glycine max 32.01 9.00e-58 217.00
IUUC-Ggo-045627 Gorilla gorilla 91.04 0.00e+00 741.00
IUUC-Hvu-047678 Hordeum vulgare 32.06 3.00e-34 139.00
IUUC-Itr-048648 Ictidomys tridecemlineatus 95.20 0.00e+00 799.00
IUUC-Kpa-049386 Komagataella pastoris 30.33 2.00e-43 169.00
IUUC-Lch-050590 Latimeria chalumnae 79.31 4.00e-162 563.00
IUUC-Lpe-051725 Leersia perrieri 32.80 3.00e-35 142.00
IUUC-Loc-051788 Lepisosteus oculatus 74.23 2.00e-179 621.00
IUUC-Lma-053082 Leptosphaeria maculans 33.71 2.00e-54 206.00
IUUC-Laf-053809 Loxodonta africana 94.71 0.00e+00 790.00
IUUC-Mcc-054998 Macaca mulatta 99.29 1.00e-167 582.00
IUUC-Meu-055929 Macropus eugenii 77.39 0.00e+00 636.00
IUUC-Mor-057003 Magnaporthe oryzae 33.63 3.00e-48 186.00
IUUC-Mtr-057921 Medicago truncatula 32.03 6.00e-57 214.00
IUUC-Mga-059449 Meleagris gallopavo 65.59 1.00e-125 442.00
IUUC-Mmr-061609 Microcebus murinus 92.07 0.00e+00 743.00
IUUC-Mdo-062564 Monodelphis domestica 85.14 0.00e+00 724.00
IUUC-Mmu-063904 Mus musculus 89.67 0.00e+00 753.00
IUUC-Mac-064716 Musa acuminata 36.16 2.00e-40 159.00
IUUC-Mpu-066617 Mustela putorius furo 94.19 0.00e+00 789.00
IUUC-Mlu-067054 Myotis lucifugus 91.67 0.00e+00 768.00
IUUC-Nfi-068467 Neosartorya fischeri 36.90 1.00e-34 140.00
IUUC-Nle-069731 Nomascus leucogenys 100.00 0.00e+00 826.00
IUUC-Opr-070297 Ochotona princeps 68.62 1.00e-87 316.00
IUUC-Ont-072558 Oreochromis niloticus 70.32 2.00e-164 571.00
IUUC-Oan-072864 Ornithorhynchus anatinus 85.96 5.00e-27 111.00
IUUC-Ocu-074415 Oryctolagus cuniculus 95.20 0.00e+00 797.00
IUUC-Oba-075759 Oryza barthii 39.47 8.00e-43 168.00
IUUC-Obr-076387 Oryza brachyantha 37.91 7.00e-45 174.00
IUUC-Ogl-077924 Oryza glaberrima 40.60 3.00e-17 82.00
IUUC-Ogu-078186 Oryza glumaepatula 34.20 9.00e-63 234.00
IUUC-Oin-079389 Oryza indica 33.71 2.00e-46 179.00
IUUC-Olo-080916 Oryza longistaminata 28.43 3.00e-16 78.20
IUUC-Ome-081471 Oryza meridionalis 38.95 3.00e-40 159.00
IUUC-Oni-082449 Oryza nivara 38.95 5.00e-40 158.00
IUUC-Opu-083210 Oryza punctata 38.95 2.00e-42 166.00
IUUC-Oru-085159 Oryza rufipogon 39.47 5.00e-43 168.00
IUUC-Osa-085926 Oryza sativa 39.86 6.00e-33 133.00
IUUC-Ola-087084 Oryzias latipes 67.59 1.00e-165 575.00
IUUC-Olu-087853 Ostreococcus lucimarinus 28.35 5.00e-39 155.00
IUUC-Oga-089008 Otolemur garnettii 93.48 0.00e+00 780.00
IUUC-Oar-089259 Ovis aries 94.46 0.00e+00 783.00
IUUC-Ptr-091112 Pan troglodytes 99.24 0.00e+00 815.00
IUUC-Pan-092303 Papio anubis 100.00 8.00e-75 271.00
IUUC-Psi-093050 Pelodiscus sinensis 82.16 0.00e+00 697.00
IUUC-Pma-094259 Petromyzon marinus 56.19 7.00e-128 450.00
IUUC-Pno-094798 Phaeosphaeria nodorum 30.89 1.00e-50 194.00
IUUC-Ppa-095477 Physcomitrella patens 31.19 1.00e-53 203.00
IUUC-Pfo-096855 Poecilia formosa 67.16 1.00e-163 569.00
IUUC-Pab-098096 Pongo abelii 99.24 0.00e+00 820.00
IUUC-Pop-098800 Populus trichocarpa 36.40 4.00e-45 175.00
IUUC-Pca-100187 Procavia capensis 92.23 3.00e-113 402.00
IUUC-Ppe-101759 Prunus persica 36.00 2.00e-43 170.00
IUUC-Pva-102438 Pteropus vampyrus 78.05 2.00e-91 329.00
IUUC-Pte-104197 Pyrenophora teres 33.33 4.00e-50 192.00
IUUC-Pyt-104421 Pyrenophora triticirepentis 33.33 1.00e-49 191.00
IUUC-Rno-106087 Rattus norvegicus 90.43 0.00e+00 760.00
IUUC-Sce-106276 Saccharomyces cerevisiae 26.80 4.00e-12 66.60
IUUC-Sha-106831 Sarcophilus harrisii 82.41 0.00e+00 690.00
IUUC-Sja-107681 Schizosaccharomyces japonicus 33.07 4.00e-57 215.00
IUUC-Spo-108294 Schizosaccharomyces pombe 34.83 6.00e-64 238.00
IUUC-Ssl-108613 Sclerotinia sclerotiorum 32.67 6.00e-52 198.00
IUUC-Smo-109008 Selaginella moellendorffii 40.90 2.00e-76 279.00
IUUC-Sit-110300 Setaria italica 29.95 2.00e-52 199.00
IUUC-Sly-111441 Solanum lycopersicum 28.94 7.00e-45 174.00
IUUC-Stu-111995 Solanum tuberosum 28.74 5.00e-45 175.00
IUUC-Sar-113196 Sorex araneus 84.03 2.00e-130 458.00
IUUC-Sbi-113790 Sorghum bicolor 40.00 2.00e-43 170.00
IUUC-Ssc-115609 Sus scrofa 94.44 0.00e+00 794.00
IUUC-Tgu-116927 Taeniopygia guttata 79.70 0.00e+00 663.00
IUUC-Tru-118164 Takifugu rubripes 68.98 4.00e-171 593.00
IUUC-Tsy-119151 Tarsius syrichta 89.90 0.00e+00 741.00
IUUC-Tni-120896 Tetraodon nigroviridis 68.25 1.00e-161 562.00
IUUC-Tca-121592 Theobroma cacao 29.37 2.00e-45 176.00
IUUC-Tre-122023 Trichoderma reesei 34.73 4.00e-49 189.00
IUUC-Tvi-122507 Trichoderma virens 35.10 4.00e-50 192.00
IUUC-Tae-125730 Triticum aestivum 33.73 1.00e-59 223.00
IUUC-Tur-126092 Triticum urartu 30.29 3.00e-34 139.00
IUUC-Tbe-127422 Tupaia belangeri 71.35 3.00e-152 531.00
IUUC-Ttr-129097 Tursiops truncatus 91.41 0.00e+00 767.00
IUUC-Vda-130014 Verticillium dahliae 36.05 4.00e-51 195.00
IUUC-Vpa-130782 Vicugna pacos 94.96 0.00e+00 792.00
IUUC-Vvi-131494 Vitis vinifera 34.65 2.00e-43 170.00
IUUC-Xtr-132165 Xenopus tropicalis 77.84 0.00e+00 629.00
IUUC-Xma-133205 Xiphophorus maculatus 67.83 3.00e-167 581.00
IUUC-Yli-134614 Yarrowia lipolytica 32.86 1.00e-59 224.00
IUUC-Zma-135861 Zea mays 34.15 4.00e-60 225.00
Created Date 25-Jun-2017

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