THE MOLECULAR-BASIS OF XERODERMA-PIGMENTOSUM

BACKGROUND. Xeroderma pigmentostrm is an extremely rave, autosomal rec essive disease characterized by a more than 1000-fold increase in nonm elanoma skin cancer. Individuals with this disease can be divided into eight complementation groups: A-G and V for variant. Each one represe nts a different genetic defect in DNA repair. OBJECTIVE. To review the molecular basis of xeroderma pigmentosum. RESULTS. Deficiencies in va rious gene products in the nucleotide excision repair pathway cause xe roderma pigmentosum in complementation groups A-G. The molecular basis of the variant group remains to be elucidated. CONCLUSIONS. Research into the genetic defects underlying xeroderma pigmentostrm have led to an increased understanding of nucleotide excision repair. (C) 1997 by the American Society for Dermatologic Surgery, Inc.