Xeroderma pigmentosum patients belonging to complementation group F and efficient liquid-holding recovery of ultraviolet damage.
A 73-year-old woman with xeroderma pigmentosum (XP), XP107TO, was assigned to complementation group F. The fibroblast of this patient showed a reduced level (19%) of unscheduled DNA synthesis (UDS) compared with normal cells. XP107TO cells were 2.3 times more hypersensitive to ultraviolet C (UVC) than normal cells and in the same range of other XPF cells. A clinical case report of 2 patients belonging to XP group F is also described; together with these clinical findings, the characteristics of XP group F are discussed. These patients manifested mild clinical symptoms, developing skin cancer in later age, although acute sun sensitivity was observed in early age (usually a few months after birth). Fibroblasts derived from these patients showed a substantially reduced level of UDS, considering its mild clinical features. To analyze the discrepancy of low UDS level and late onset of skin cancer in XPF cells, we examined the UV sensitivity under density-inhibited condition using XPF cells. XP cells belonging to complementation group F showed marked enhancement of UV survival when they were held in a density-inhibited condition for 1-4 d after UV irradiation. The enhancement was also observed in normal and XP group A cells, but the recovery in these cell lines was less than that in XP group F cells. XP group F cells have been shown to possess slow and long-lasting excision repair capacity and this type of repair might be stimulated by holding through time of the repair process within one cell cycle.
Nishigori
C
C
Department of Dermatology, Faculty of Medicine, Kyoto University, Japan.
Fujisawa
H
H
Uyeno
K
K
Kawaguchi
T
T
Takebe
H
H
D000328:Adult; D000368:Aged; D002280:Basal Cell Carcinoma / Q000235:genetics / Q000473:pathology; D002294:Carcinoma, Squamous Cell / Q000235:genetics / Q000473:pathology; D002470:Cell Survival / Q000528:radiation effects; D004247:DNA / Q000096:biosynthesis / Q000528:radiation effects; D004260:DNA Repair; D005260:Female; D005347:Fibroblasts / Q000528:radiation effects; D005816:Genetic Complementation Test; D006801:Humans; D007636:Keratoacanthoma / Q000235:genetics / Q000473:pathology; D008297:Male; D008875:Middle Aged; D012878:Skin Neoplasms / Q000235:genetics* / Q000473:pathology; D014466:Ultraviolet Rays; D014983:Xeroderma Pigmentosum / Q000235:genetics*
D004247:DNA