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    An Xpd mouse model for the combined xeroderma pigmentosum/Cockayne syndrome exhibiting both cancer predisposition and segmental progeria.

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    Authors
    Andressoo, Jaan-Olle
    Mitchell, James R
    Wit, Jan de
    Hoogstraten, Deborah
    Volker, Marcel
    Toussaint, Wendy
    Speksnijder, Ewoud
    Beems, Rudolf B
    Steeg, Harry van
    Jans, Judith
    Zeeuw, Chris I de
    Jaspers, Nicolaas G J
    Raams, Anja
    Lehmann, Alan R
    Vermeulen, Wim
    Hoeijmakers, Jan H J
    Horst, Gijsbertus T J van der
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    Article
    Language
    en
    
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    Title
    An Xpd mouse model for the combined xeroderma pigmentosum/Cockayne syndrome exhibiting both cancer predisposition and segmental progeria.
    Publiekssamenvatting
    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 cancer-prone NER model to date, and we postulate an unusual NER dysfunction that is likely responsible for this susceptibility. XPCS mice also displayed symptoms of segmental progeria, including cachexia and progressive loss of germinal epithelium. Like CS fibroblasts, XPCS and TTD fibroblasts from human and mouse showed evidence of defective repair of oxidative DNA lesions that may underlie these segmental progeroid symptoms.
    DOI
    10.1016/j.ccr.2006.05.027
    PMID
    16904611
    URI
    http://hdl.handle.net/10029/5565
    ae974a485f413a2113503eed53cd6c53
    10.1016/j.ccr.2006.05.027
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