UniProt Accession

 ERCC8_HUMAN; Q13216; B2RB64; Q6FHX5; Q96GB9 

Genbank Protein ID

 U28413; CR536563; BT020021; AY213194; AK314511; CH471123; BC009793 

Genbank Nucleotide ID

 AAA82605.1; CAG38800.1; AAV38824.1; AAO21128.1; BAG37111.1; EAW55004.1; AAH09793.1 

Protein Name

 DNA excision repair protein ERCC-8 

Protein Synonyms/Alias

 Cockayne syndrome WD repeat protein CSA 

Gene Name

 ERCC8 

Gene Synonyms/Alias

 CKN1; CSA 

Created Date

 July 27, 2013 

Organism

 Homo sapiens (Human) 

NCBI Taxa ID

 9606 

Lysine Modification

Position	Peptide	Type	References
125	SSFDKTLKVWDTNTL	ubiquitination	[1]
167	AVGTRGPKVQLCDLK	ubiquitination	[1]
212	ASADSRVKLWDVRRA	ubiquitination	[1]
233	LDQHNGKKSQAVESA	ubiquitination	[2]
247	ANTAHNGKVNGLCFT	ubiquitination	[1]
286	NTLVNYGKVCNNSKK	ubiquitination	[1]

Reference

 [1] Refined preparation and use of anti-diglycine remnant (K-ε-GG) antibody enables routine quantification of 10,000s of ubiquitination sites in single proteomics experiments.
 Udeshi ND, Svinkina T, Mertins P, Kuhn E, Mani DR, Qiao JW, Carr SA.
 Mol Cell Proteomics. 2013 Mar;12(3):825-31. [PMID: 23266961]
 [2] Systematic and quantitative assessment of the ubiquitin-modified proteome.
 Kim W, Bennett EJ, Huttlin EL, Guo A, Li J, Possemato A, Sowa ME, Rad R, Rush J, Comb MJ, Harper JW, Gygi SP.
 Mol Cell. 2011 Oct 21;44(2):325-40. [PMID: 21906983] 

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. 

Sequence Annotation

 REPEAT 41 72 WD 1.
 REPEAT 97 128 WD 2.
 REPEAT 184 215 WD 3.
 REPEAT 243 273 WD 4.
 REPEAT 332 362 WD 5.
 MOD_RES 390 390 Phosphoserine.
 MOD_RES 391 391 Phosphoserine.
 MOD_RES 392 392 Phosphoserine.  

Keyword

 3D-structure; Alternative splicing; Cockayne syndrome; Complete proteome; Deafness; Disease mutation; DNA damage; DNA repair; Dwarfism; Nucleus; Phosphoprotein; Polymorphism; Reference proteome; Repeat; Ubl conjugation pathway; WD repeat. 

Sequence Source

 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 

Protein Length

 396 AA 

Protein Sequence

Gene Ontology

 GO:0031464; C:Cul4A-RING ubiquitin ligase complex; IDA:UniProtKB.
 GO:0016363; C:nuclear matrix; IDA:MGI.
 GO:0005654; C:nucleoplasm; TAS:Reactome.
 GO:0000109; C:nucleotide-excision repair complex; IDA:MGI.
 GO:0032403; F:protein complex binding; IDA:UniProtKB.
 GO:0045739; P:positive regulation of DNA repair; IMP:UniProtKB.
 GO:0043161; P:proteasomal ubiquitin-dependent protein catabolic process; IDA:UniProtKB.
 GO:0051865; P:protein autoubiquitination; IDA:UniProtKB.
 GO:0000209; P:protein polyubiquitination; IDA:UniProtKB.
 GO:0006979; P:response to oxidative stress; IDA:UniProtKB.
 GO:0009411; P:response to UV; IDA:UniProtKB.
 GO:0010165; P:response to X-ray; IEA:Compara.
 GO:0006283; P:transcription-coupled nucleotide-excision repair; IMP:UniProtKB. 

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. 

Pfam

 PF00400; WD40 

SMART

 SM00320; WD40 

PROSITE

 PS00678; WD_REPEATS_1
 PS50082; WD_REPEATS_2
 PS50294; WD_REPEATS_REGION 

PRINTS

 PR00320; GPROTEINBRPT.