title: Correction of the ultraviolet light induced DNA-repair defect in xeroderma pigmentosum cells by electroporation of a normal human endonuclease.
abstract: Cells from patients with xeroderma pigmentosum, complementation group A (XPA), are known to be defective in repair of pyrimidine dimers and other forms of damage produced by 254-nm ultraviolet (UVC) radiation. We have isolated a DNA endonuclease, pI 7.6, from the chromatin of normal human lymphoblastoid cells which recognizes damage produced by UVC light, and have introduced this endonuclease into UVC-irradiated XPA cells in culture to determine whether it can restore their markedly deficient DNA repair-related unscheduled DNA synthesis (UDS). Introduction of the normal endonuclease, which recognizes predominantly pyrimidine dimers, but not the corresponding XPA endonuclease into UVC-irradiated XPA cells restored their levels of UDS to approximately 80% of normal values. Electroporation of both the normal and the XPA endonuclease into normal human cells increases UDS in normal cells to higher than normal values. These results indicate that the normal endonuclease can restore UDS in UVC-irradiated XPA cells. They also indicate that XPA cells have an endonuclease capable of increasing the efficiency of repair of UVC damage in normal cells.
authors.affiliation: Department of Pathology, UMDNJ-New Jersey Medical School, Newark 07103.
authors.forename: G J
authors.initials: GJ
authors.lastname: Tsongalis
authors.forename: W C
authors.initials: WC
authors.lastname: Lambert
authors.forename: M W
authors.initials: MW
authors.lastname: Lambert
mesh_terms: D002461:Cell Line, Transformed; D004247:DNA / Q000096:biosynthesis; D004260:DNA Repair / Q000187:drug effects / Q000528:radiation effects*; D003850:Deoxyribonuclease I / Q000627:therapeutic use*; D006801:Humans; D014466:Ultraviolet Rays; D014983:Xeroderma Pigmentosum / Q000188:drug therapy* / Q000201:enzymology / Q000235:genetics
chemical_list: D004247:DNA; D003850:Deoxyribonuclease I