Reduced DNA repair in progeria cells and effects of gamma-ray irradiation on UV-induced unscheduled DNA synthesis in normal and progeria cells

Mutat Res. 1991 Jan;256(1):59-66. doi: 10.1016/0921-8734(91)90034-9.


A reduction in the amount of UV-induced unscheduled DNA synthesis (UDS), and reduced cell survival and host-cell reactivation against UV exposure in Hutchinson-Gilford progeria syndrome cell strains were shown. UV-induced UDS in 4 progeria cell strains was 33-50% of the normal level. A similar reduction in the UV-induced UDS in normal cells was caused by gamma-ray irradiation to the cells before UV irradiation. The dose of gamma-rays required to cause a reduction in UDS of normal cells to the level of progeria cells was 40 Gy and the reduction was reversible after 2 days. In progeria cells, gamma-ray irradiation further reduced UDS with a lower gamma-ray dose required than in normal cells, and the reduction was also reversible but with less relative recovery than in normal cells. The presence of a 'built-in' defect in progeria cells responsible for the reduced DNA-repair capacity was suggested, and such defect may share a common mechanism with the reduction of UV-induced UDS in normal cells caused by gamma-ray irradiation.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aging
  • Cell Survival / genetics
  • Cell Survival / radiation effects*
  • Cells, Cultured
  • Child
  • Child, Preschool
  • DNA / biosynthesis*
  • DNA Repair* / radiation effects
  • Dose-Response Relationship, Radiation
  • Female
  • Gamma Rays
  • Humans
  • Kinetics
  • Male
  • Middle Aged
  • Progeria / genetics*
  • Ultraviolet Rays


  • DNA