Article
UVB radiation-induced cancer predisposition in Cockayne syndrome group A (Csa) mutant mice.
DNA repair - 28 Feb 2002
van der Horst Gijsbertus T J, Meira Lisiane, Gorgels Theo G M F, de Wit Jan, Velasco-Miguel Susana, Richardson James A, Kamp Yvonne, Vreeswijk Maaike P G, Smit Bep, Bootsma Dirk, Hoeijmakers Jan H J, Friedberg Errol C
Abstract excerpt
Cockayne syndrome (CS) is an inherited photosensitive neurodevelopmental disorder caused by a specific defect in the transcription-coupled repair (TCR) sub-pathway of NER. Remarkably, despite their DNA repair deficiency, CS patients do not develop skin cancer. Here, we present a mouse model for CS complementation group A. Like cells from CS-A patients, Csa-/- mouse embryonic fibroblasts (MEFs): (i) are...
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