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Xeroderma Pigmentosum (XP) is a rare hereditary disease characterized by increased sensitivity to sunlight and high incidence of skin cancers. These phenotypical features could be attributed to a deficiency in the repair of UV-light-induced DNA lesions. Thereafter, the proteins mutated in either of the 7 XP complementation groups (XPA-XPG) were cloned and shown to be directly involved in a DNA repair pathway, namely the Nucleotide Excision Repair pathway.
Now, using cultured cells issued from XP patients, researchers have developed a skin model for XP. As reported in the last week's issue of PNAS (Bernerd et al., Proc Natl Acad Sci U S A 2001 Jul 3; 98(14):7817-7822), the in vitro reconstituted XP skin presents both a high proliferation rate of keratinocytes and epidermal invasion within dermal skin parts which are characteristic of early steps of skin neoplasia. Thus, this in vitro skin model will not only open new research opportunities for XP therapy, but could also represent a powerful tool for photoaging and photocarcinogenesis research. CONTACT Thierry Magnaldo
CNRS UPR 2169
7, rue Guy Môquet
94801 Villejuif, France
e-mail magnaldo@infobiogen.fr
Message posted by: Ulrike Sattler
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