The molecular basis of Xeroderma pigmentosum

BACKGROUND. Xeroderma pigmentostrm is an extremely rave, autosomal recessive disease characterized by a more than 1000-fold increase in nonmelanoma skin cancer. Individuals with this disease can be divided into eight complementation groups: A-G and V for variant. Each one represents a different genetic defect in DNA repair. OBJECTIVE. To review the molecular basis of xeroderma pigmentosum. RESULTS. Deficiencies in various gene products in the nucleotide excision repair pathway cause xeroderma 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.