Article
An Xpd mouse model for the combined xeroderma pigmentosum/Cockayne syndrome exhibiting both cancer predisposition and segmental progeria.
Cancer cell - 1 Aug 2006
Andressoo Jaan-Olle, Mitchell James R, de Wit Jan, Hoogstraten Deborah, Volker Marcel, Toussaint Wendy, Speksnijder Ewoud, Beems Rudolph B, van Steeg Harry, Jans Judith, de Zeeuw Chris I, Jaspers Nicolaas G J, Raams Anja, Lehmann Alan R, Vermeulen Wim, Hoeijmakers Jan H J, van der Horst Gijsbertus T J
Abstract excerpt
Inborn defects in nucleotide excision DNA repair (NER) can paradoxically result in elevated cancer incidence (xeroderma pigmentosum [XP]) or segmental progeria without cancer predisposition (Cockayne syndrome [CS] and trichothiodystrophy [TTD]). We report generation of a knockin mouse model for the combined disorder XPCS with a G602D-encoding mutation in the Xpd helicase gene. XPCS mice are the most skin...
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