Since it has been shown previously that expression of NER genes such as XPC and XPE can be transactivated by p53 after UV-radiation30 the present data confirm the notion that NER genes can be differentially expressed and that they may be associated with the clinical phenotype not only in XP patients but also in individuals with no known defect in DNA repair but increased skin cancer risk. Here, NR1H2 is linked to xeroderma pigmentosum.