Article
An Xpb mouse model for combined xeroderma pigmentosum and cockayne syndrome reveals progeroid features upon further attenuation of DNA repair.
Molecular and cellular biology - 1 Mar 2009
Andressoo Jaan-Olle, Weeda Geert, de Wit Jan, Mitchell James R, Beems Rudolf B, van Steeg Harry, van der Horst Gijsbertus T J, Hoeijmakers Jan H
Abstract excerpt
Patients carrying mutations in the XPB helicase subunit of the basal transcription and nucleotide excision repair (NER) factor TFIIH display the combined cancer and developmental-progeroid disorder xeroderma pigmentosum/Cockayne syndrome (XPCS). Due to the dual transcription repair role of XPB and the absence of animal models, the underlying molecular mechanisms of XPB(XPCS) are largely uncharacterized. Here we...
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