title: Reduced DNA-repair capacity in cells originating from a progeria patient.
abstract: A Chinese boy was identified to be suffering from progeria (Hutchinson-Gilford syndrome), the first case of the disease ever reported in China. Cells originating from the patient had a reduced amount of unscheduled DNA synthesis after irradiation with ultraviolet light (UV). The fractions of the progeria cells surviving against UV irradiation measured by colony-forming ability, and the host-cell reactivation capacity of the progeria cells, measured by the plaque formation of UV-irradiated herpes simplex virus were lower than those measured in normal cells. The progeria cells appear to have a reduced capacity to repair UV excision damage.
authors.affiliation: Department of Experimental Radiology, Kyoto University, Japan.
authors.forename: S M
authors.initials: SM
authors.lastname: Wang
authors.forename: C K
authors.initials: CK
authors.lastname: Nishigori
authors.forename: J M
authors.initials: JM
authors.lastname: Zhang
authors.forename: H
authors.initials: H
authors.lastname: Takebe
mesh_terms: D002478:Cells, Cultured; D002648:Child; D004260:DNA Repair; D004261:DNA Replication / Q000528:radiation effects; D006801:Humans; D008297:Male; D011371:Progeria / Q000235:genetics* / Q000473:pathology; D012016:Reference Values; D012867:Skin / Q000166:cytology / Q000473:pathology* / Q000528:radiation effects; D014466:Ultraviolet Rays