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Protein

DNA excision repair protein ERCC-8

Gene

Ercc8

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -Experimental evidence at transcript leveli

Functioni

Involved in transcription-coupled nucleotide excision 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. It is the substrate-recognition component of the CSA complex (DCX(ERCC8) complex) which 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.By similarity

Miscellaneous

Ercc8 deficient mice develop normally, but show a loss of retinal photoreceptors after 4 months of age, and enhanced UV-sensitivity.

Pathwayi: protein ubiquitination

This protein is involved in the pathway protein ubiquitination, which is part of Protein modification.
View all proteins of this organism that are known to be involved in the pathway protein ubiquitination and in Protein modification.

GO - Molecular functioni

GO - Biological processi

Keywordsi

Biological processDNA damage, DNA repair, Ubl conjugation pathway

Enzyme and pathway databases

ReactomeiR-MMU-6781823 Formation of TC-NER Pre-Incision Complex
R-MMU-6782135 Dual incision in TC-NER
R-MMU-6782210 Gap-filling DNA repair synthesis and ligation in TC-NER
R-MMU-8951664 Neddylation
UniPathwayiUPA00143

Names & Taxonomyi

Protein namesi
Recommended name:
DNA excision repair protein ERCC-8
Alternative name(s):
Cockayne syndrome WD repeat protein CSA homolog
Gene namesi
Name:Ercc8
Synonyms:Ckn1, Csa
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 13

Organism-specific databases

MGIiMGI:1919241 Ercc8

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Nucleus

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00000509711 – 397DNA excision repair protein ERCC-8Add BLAST397

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei391PhosphoserineBy similarity1
Modified residuei392PhosphoserineBy similarity1
Modified residuei393PhosphoserineBy similarity1

Keywords - PTMi

Phosphoprotein

Proteomic databases

MaxQBiQ8CFD5
PaxDbiQ8CFD5
PRIDEiQ8CFD5

PTM databases

PhosphoSitePlusiQ8CFD5

Expressioni

Gene expression databases

BgeeiENSMUSG00000021694
ExpressionAtlasiQ8CFD5 baseline and differential
GenevisibleiQ8CFD5 MM

Interactioni

Subunit structurei

Part of the CSA complex (DCX(ERCC8) complex), a DCX E3 ubiquitin-protein ligase complex containing ERCC8, RBX1, DDB1 and CUL4A; the CSA complex interacts with RNA polymerase II; upon UV irradiation it interacts with the COP9 signalosome and preferentially with the hyperphosphorylated form of RNA polymerase II. Interacts with ERCC6 and KIAA1530/UVSSA. Interacts with a subunit of RNA polymerase II TFIIH. Interacts with DDB1.By similarity

Protein-protein interaction databases

BioGridi215079, 10 interactors
STRINGi10090.ENSMUSP00000059211

Structurei

3D structure databases

ProteinModelPortaliQ8CFD5
SMRiQ8CFD5
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Repeati41 – 81WD 1Add BLAST41
Repeati97 – 137WD 2Add BLAST41
Repeati184 – 224WD 3Add BLAST41
Repeati243 – 282WD 4Add BLAST40
Repeati332 – 371WD 5Add BLAST40

Keywords - Domaini

Repeat, WD repeat

Phylogenomic databases

eggNOGiKOG4283 Eukaryota
ENOG410Z0MQ LUCA
GeneTreeiENSGT00390000009065
HOGENOMiHOG000248233
HOVERGENiHBG005409
InParanoidiQ8CFD5
KOiK10570
OMAiTMLRGHY
OrthoDBiEOG091G09HW
PhylomeDBiQ8CFD5
TreeFamiTF101237

Family and domain databases

Gene3Di2.130.10.10, 1 hit
InterProiView protein in InterPro
IPR020472 G-protein_beta_WD-40_rep
IPR015943 WD40/YVTN_repeat-like_dom_sf
IPR001680 WD40_repeat
IPR019775 WD40_repeat_CS
IPR017986 WD40_repeat_dom
IPR036322 WD40_repeat_dom_sf
PfamiView protein in Pfam
PF00400 WD40, 4 hits
PRINTSiPR00320 GPROTEINBRPT
SMARTiView protein in SMART
SM00320 WD40, 5 hits
SUPFAMiSSF50978 SSF50978, 1 hit
PROSITEiView protein in PROSITE
PS00678 WD_REPEATS_1, 2 hits
PS50082 WD_REPEATS_2, 5 hits
PS50294 WD_REPEATS_REGION, 1 hit

Sequencei

Sequence statusi: Complete.

Q8CFD5-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MLSFLSARQS GLEDPLRLRR AQSTRRVLGL ELNKDRDVER IHGSGVNTLD
60 70 80 90 100
IEPVEGRYML SGGSDGVVVL YDLENASRQP HYTCKAVCSV GRSHPDVHKY
110 120 130 140 150
SVETVQWYPH DTGMFTSSSF DKTLKVWDTN TLQAADVFNF EETVYSHHMS
160 170 180 190 200
PAATKHCLVA VGTRGPKVQL CDLKSGSCSH ILQGHRQEIL AVSWSPRHDY
210 220 230 240 250
ILATASADSR VKLWDVRRAS GCLLTLDQHN GKKSQAAESA NTAHNGKVNG
260 270 280 290 300
LCFTSDGLHL LTIGTDNRMR LWNSSSGDNT LVNYGKVCND SRKGLQFAVS
310 320 330 340 350
CGCSSEFVFV PHGSTIAVYA VHSGERLAML KGHYKSVDCC VFQPNFQELY
360 370 380 390
SGSRDCNILA WVPPSYEPVP DDDDEAPAKS QLNPAFADAW SSSDEDG
Length:397
Mass (Da):43,689
Last modified:June 7, 2005 - v2
Checksum:i1B35EA61A00A611B
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti90V → I in AAH37200 (PubMed:15489334).Curated1
Sequence conflicti292R → M in BAB27039 (PubMed:16141072).Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AJ427222 mRNA Translation: CAD20255.1
AK010576 mRNA Translation: BAB27039.1
AK157175 mRNA Translation: BAE33989.1
BC037200 mRNA Translation: AAH37200.1
CCDSiCCDS26761.1
RefSeqiNP_082318.2, NM_028042.3
UniGeneiMm.212208

Genome annotation databases

EnsembliENSMUST00000054835; ENSMUSP00000059211; ENSMUSG00000021694
GeneIDi71991
KEGGimmu:71991
UCSCiuc007rux.1 mouse

Similar proteinsi

Entry informationi

Entry nameiERCC8_MOUSE
AccessioniPrimary (citable) accession number: Q8CFD5
Secondary accession number(s): Q3U066, Q8VDC4, Q9CWK7
Entry historyiIntegrated into UniProtKB/Swiss-Prot: June 7, 2005
Last sequence update: June 7, 2005
Last modified: April 25, 2018
This is version 118 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome
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Main funding by: National Institutes of Health