|Other Names||DNA excision repair protein ERCC-8, Cockayne syndrome WD repeat protein CSA, ERCC8, CKN1, CSA|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Function||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.|
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This gene encodes a WD repeat protein, which interactswith Cockayne syndrome type B (CSB) protein and with p44 protein, asubunit of the RNA polymerase II transcription factor IIH.Mutations in this gene have been identified in patients withhereditary disease Cockayne syndrome (CS). CS cells are abnormallysensitive to ultraviolet radiation and are defective in the repairof transcriptionally active genes.
Briggs, F.B., et al. Am. J. Epidemiol. 172(2):217-224(2010)Guillem, V.M., et al. Am. J. Hematol. 85(7):482-486(2010)Monsees, G.M., et al. Breast Cancer Res. Treat. (2010) In press :Kamenisch, Y., et al. J. Exp. Med. 207(2):379-390(2010)Laugel, V., et al. Hum. Mutat. 31(2):113-126(2010)
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