UVs syndrome: establishment and characterization of fibroblastic cell lines transformed with simian virus 40 DNA

Ultraviolet-sensitive syndrome (UVsS) is a newly established photosensitive disorder. Patients with UVsS showed mild clinical manifestations similar to classical types of xeroderma pigmentosum, and had biochemical phenotypes of Cockayne syndrome but not those of xeroderma pigmentosum. Fibroblasts from a UVsS patient were treated with simian virus 40 DNA containing the large T antigen with a defective origin of DNA replication to establish a transformed cell line. We obtained two independent transformed cell lines (Kps3SVY and Kps3SVI3) and report their initial characterization. These cells showed the same pattern in variable number of tandem repeat analyses as a primary fibroblast cell strain, Kps3, and retain the UVsS phenotype as demonstrated by increased UV sensitivity (three to four times more sensitive to UV than normal cells) and by reduced recovery of RNA synthesis after UV irradiation (20% - 30% of that of normal cells). These cells, however, showed different phenotypes as regards plating efficiency, doubling time, and transfection efficiency in spite of the fact that the same method was used to transform the cells. Kps3SVY cells were closer in phenotype to Kps3 cells than Kps3SVI3 cells. As a variable number of tandem repeat analyses also showed that Kps3SVI3 cells have lost one of the two alleles in some chromosomes, this may explain the different phenotypes between Kps3SVY and Kps3SVI3 cells. Moreover, these cells were distinct from cells with Cockayne syndrome group A or B. Thus, these cell lines provide the opportunity to conduct transfection studies on cells with the UVsS defect in DNA repair and transcription.