Article
DNA repair-deficient Xpa and Xpa/p53+/- knock-out mice: nature of the models.
Toxicologic pathology - 1 Jan 2001
van Steeg H, de Vries A, van Oostrom CTh, van Benthem J, Beems R B, van Kreijl C F
Abstract excerpt
Xeroderma pigmentosum (XP) is a rare autosomal recessive disease in which repair of ultraviolet (UV)-induced DNA damage is impaired or is totally absent due to mutations in genes controlling the DNA repair pathway known as nucleotide excision repair (NER). XP is characterized, in part, by extreme sensitivity of the skin to sunlight, and XP patients have a more than 1000-fold increased risk of developing cancer at...
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