Article
Defective transcription-coupled repair in Cockayne syndrome B mice is associated with skin cancer predisposition.
Cell - 2 May 1997
van der Horst G T, van Steeg H, Berg R J, van Gool A J, de Wit J, Weeda G, Morreau H, Beems R B, van Kreijl C F, de Gruijl F R, Bootsma D, Hoeijmakers J H
Abstract excerpt
A mouse model for the nucleotide excision repair disorder Cockayne syndrome (CS) was generated by mimicking a truncation in the CSB(ERCC6) gene of a CS-B patient. CSB-deficient mice exhibit all of the CS repair characteristics: ultraviolet (UV) sensitivity, inactivation of transcription-coupled repair, unaffected global genome repair, and inability to resume RNA synthesis after UV exposure. Other CS features...
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