期刊
CANCER CELL
卷 10, 期 2, 页码 121-132出版社
CELL PRESS
DOI: 10.1016/j.ccr.2006.05.027
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资金
- NIA NIH HHS [1P01 AG17242-02] Funding Source: Medline
- NIEHS NIH HHS [1U01 ES011044] Funding Source: Medline
- Medical Research Council [G0300662B] Funding Source: researchfish
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.
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