Selective defects in GG-NER, resulting from mutations in the XPC and XPE (also termed UV-DDB2) genes encoding GG-NER-specific damage recognition proteins, cause xeroderma pigmentosum (XP), a photosensitivity syndrome characterized by UV-hypersensitivity, pigmentation abnormalities and UV-induced skin cancer predisposition [11], [12]. Here, XPC is linked to xeroderma pigmentosum.