AC T04550
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ID T04550
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DT 25.06.2001 (created); mpr.
DT 19.09.2011 (updated); pum.
CO Copyright (C), QIAGEN.
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FA CSB
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SY CKN2; COFS; CSB; ERCC6; excision repair cross-complementing rodent repair deficiency, complementation group 6; RAD26.
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OS human, Homo sapiens
OC eukaryota; animalia; metazoa; chordata; vertebrata; tetrapoda; mammalia; eutheria; primates
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GE G004036 ERCC6; HGNC: ERCC6.
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SF contains seven domains characteristic for DNA helicases [1];
SF does not appear to be a helicase (despite helicase-like domains), but rather a member of the SWI2/SNF2 family of ATPases [4];
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FF interacts with Pol II molecules engaged in ternary complexes containing DNA and nascent RNA;
FF is a DNA-activated ATPase, and hydrolysis of the ATP beta-gamma phosphoanhydride bond is required for the formation of stable POL II-CSB-DNA-RNA complex;
FF mutations are associated with Cockayne's syndrome [2];
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IN T04145 WSTF; human, Homo sapiens.
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DR TRANSPATH: MO000028387.
DR PATHODB: MT010882.
DR PATHODB: MT010883.
DR PATHODB: MT010887.
DR PATHODB: MT010888.
DR PATHODB: MT010889.
DR PATHODB: MT010890.
DR PATHODB: MT010891.
DR PATHODB: MT010892.
DR PATHODB: MT010893.
DR PATHODB: MT010894.
DR PATHODB: MT010895.
DR PATHODB: MT010896.
DR PATHODB: MT010897.
DR PATHODB: MT010898.
DR PATHODB: MT010899.
DR PATHODB: MT010900.
DR PATHODB: MT010901.
DR PATHODB: MT010902.
DR PATHODB: MT010903.
DR PATHODB: MT010904.
DR PATHODB: MT010905.
DR PATHODB: MT010906.
DR PATHODB: MT010907.
DR UniProtKB: Q03468;
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RN [1]; RE0016352.
RX PUBMED: 1339317.
RA Troelstra C., van Gool A., de Wit J., Vermeulen W., Bootsma D., Hoeijmakers J. H.
RT ERCC6, a member of a subfamily of putative helicases, is involved in Cockayne's syndrome and preferential repair of active genes.
RL Cell 71:939-953 (1992).
RN [2]; RE0016353.
RX PUBMED: 9372911.
RA Tantin D., Kansal A., Carey M.
RT Recruitment of the putative transcription-repair coupling factor CSB/ERCC6 to RNA polymerase II elongation complexes
RL Mol. Cell. Biol. 17:6803-6814 (1997).
RN [3]; RE0016378.
RX PUBMED: 10564257.
RA Brosh RM J. r., Balajee A. S., Selzer R. R., Sunesen M., Proietti De Santis L., Bohr V. A.
RT The ATPase domain but not the acidic region of Cockayne syndrome group B gene product is essential for DNA repair.
RL Mol. Biol. Cell 10:3583-3594 (1999).
RN [4]; RE0016398.
RX PUBMED: 8999876.
RA Selby C. P., Sancar A.
RT 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.
RL J. Biol. Chem. 272:1885-1890 (1997).
RN [5]; RE0047702.
RX PUBMED: 12419226.
RA Bradsher J., Auriol J., Proietti De Santis L., Iben S., Vonesch J. L., Grummt I., Egly J. M.
RT CSB is a component of RNA pol I transcription.
RL Mol. Cell 10:819-829 (2002).
RN [6]; RE0071073.
RX PUBMED: 16603771.
RA Cavellan E., Asp P., Percipalle P., Farrants A. K.
RT The WSTF-SNF2h chromatin remodeling complex interacts with several nuclear proteins in transcription.
RL J. Biol. Chem. 281:16264-16271 (2006).
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