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Excision repair cross-complementation group 6
Available structures
PDB Ortholog search: PDBe, RCSB
External IDs OMIM609413 MGI1100494 HomoloGene133552 GeneCards: ERCC6 Gene
Species Human Mouse
Entrez 2074 319955
Ensembl ENSG00000225830 ENSMUSG00000054051
UniProt Q03468 A3KMN2
RefSeq (mRNA) NM_000124 NM_001081221
RefSeq (protein) NP_000115 NP_001074690
Location (UCSC) Chr 10:
50.72 – 50.75 Mb
Chr 14:
32.51 – 32.58 Mb
PubMed search [1] [2]

DNA excision repair protein ERCC-6 is a protein that in humans is encoded by the ERCC6 gene.[1][2][3]

Having two copies of a mutated ERCC6 causes Cockayne syndrome, type II.

The ERCC6 protein is a DNA-binding protein important in transcription-coupled excision repair. The protein has ATP-stimulated ATPase activity; there are contradictory publications reporting presence or absence of helicase activity. The protein appears to interact with several transcription and excision repair proteins, and may promote complex formation at repair sites.[3]


ERCC6 has been shown to interact with P53.[4][5]


  1. ^ Troelstra C, van Gool A, de Wit J, Vermeulen W, Bootsma D, Hoeijmakers JH (January 1993). "ERCC6, a member of a subfamily of putative helicases, is involved in Cockayne's syndrome and preferential repair of active genes". Cell 71 (6): 939–53. doi:10.1016/0092-8674(92)90390-X. PMID 1339317. 
  2. ^ Muftuoglu M, de Souza-Pinto NC, Dogan A, Aamann M, Stevnsner T, Rybanska I, Kirkali G, Dizdaroglu M, Bohr VA (March 2009). "Cockayne Syndrome Group B Protein Stimulates Repair of Formamidopyrimidines by NEIL1 DNA Glycosylase". J Biol Chem 284 (14): 9270–9. doi:10.1074/jbc.M807006200. PMC 2666579. PMID 19179336. 
  3. ^ a b "Entrez Gene: ERCC6 excision repair cross-complementing rodent repair deficiency, complementation group 6". 
  4. ^ Wang, X W; Yeh H; Schaeffer L; Roy R; Moncollin V; Egly J M; Wang Z; Freidberg E C; Evans M K; Taffe B G (June 1995). "p53 modulation of TFIIH-associated nucleotide excision repair activity". Nat. Genet. (UNITED STATES) 10 (2): 188–95. doi:10.1038/ng0695-188. ISSN 1061-4036. PMID 7663514. 
  5. ^ Yu, A; Fan H Y; Liao D; Bailey A D; Weiner A M (May 2000). "Activation of p53 or loss of the Cockayne syndrome group B repair protein causes metaphase fragility of human U1, U2, and 5S genes". Mol. Cell (UNITED STATES) 5 (5): 801–10. doi:10.1016/S1097-2765(00)80320-2. ISSN 1097-2765. PMID 10882116. 

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