Genomes and Genes
Gene Symbol: Ercc8
Description: ERCC excision repair 8, CSA ubiquitin ligase complex subunit
Alias: Ckn1, DNA excision repair protein ERCC-8, Cockayne syndrome 1 (classical), excision repair cross-complementation group 8, excision repair cross-complementing rodent repair deficiency, complementation group 8, excision repaiross-complementing rodent repair deficiency, complementation group 8
- Guthrie O. Dynamic compartmentalization of DNA repair proteins within spiral ganglion neurons in response to noise stress. Int J Neurosci. 2012;122:757-66 pubmed publisher..The combined results suggest that in response to noise stress and/or otoprotective therapy, spiral ganglion neurons may selectively remodel compartmentalized DNA repair proteins. ..
- Bertola D, Cao H, Albano L, Oliveira D, Kok F, Marques Dias M, et al. Cockayne syndrome type A: novel mutations in eight typical patients. J Hum Genet. 2006;51:701-5 pubmed..b>CKN1 is the gene responsible for CS-A, whose mutations disrupt the transcription-coupled repair system of the actively ..
- Selby C, Sancar A. Human transcription-repair coupling factor CSB/ERCC6 is a DNA-stimulated ATPase but is not a helicase and does not disrupt the ternary transcription complex of stalled RNA polymerase II. J Biol Chem. 1997;272:1885-90 pubmed..CSB is a DNA-binding protein, and it also binds to XPA, TFIIH, and the p34 subunit of TFIIE. These interactions are likely to play a role in recruiting repair proteins to ternary complexes formed at damage sites. ..
- D Errico M, Parlanti E, Teson M, Degan P, Lemma T, Calcagnile A, et al. The role of CSA in the response to oxidative DNA damage in human cells. Oncogene. 2007;26:4336-43 pubmed..These findings support the hypothesis that defective repair of oxidative DNA damage is involved in the clinical features of CS patients. ..
- de Waard H, de Wit J, Andressoo J, van Oostrom C, Riis B, Weimann A, et al. Different effects of CSA and CSB deficiency on sensitivity to oxidative DNA damage. Mol Cell Biol. 2004;24:7941-8 pubmed..We suggest that the CSA and CSB proteins in part perform separate roles in different DNA damage response pathways. ..
- Groisman R, Polanowska J, Kuraoka I, Sawada J, Saijo M, Drapkin R, et al. The ubiquitin ligase activity in the DDB2 and CSA complexes is differentially regulated by the COP9 signalosome in response to DNA damage. Cell. 2003;113:357-67 pubmed..Knockdown of CSN with RNA interference leads to defects in NER. These results suggest that the distinct UV response of the DDB2 and CSA complexes is involved in diverse mechanisms of NER. ..
- Kamiuchi S, Saijo M, Citterio E, de Jager M, Hoeijmakers J, Tanaka K. Translocation of Cockayne syndrome group A protein to the nuclear matrix: possible relevance to transcription-coupled DNA repair. Proc Natl Acad Sci U S A. 2002;99:201-6 pubmed..The hydrogen peroxide-induced translocation of CSA was also CSB dependent. These findings establish a link between TCR and the nuclear matrix mediated by CSA. ..
- Fousteri M, Vermeulen W, Van Zeeland A, Mullenders L. Cockayne syndrome A and B proteins differentially regulate recruitment of chromatin remodeling and repair factors to stalled RNA polymerase II in vivo. Mol Cell. 2006;23:471-82 pubmed..These results give insight into the nature and order of molecular events that take place during TCR in the context of chromosomal DNA. ..