Reduced superoxide dismutase activity in xeroderma pigmentosum fibroblasts

J Invest Dermatol. 1989 Oct;93(4):506-10. doi: 10.1111/1523-1747.ep12284060.

Abstract

This study was performed in order to assess the possible protective effect of superoxide dismutase (SOD) on ultraviolet (UV) damage in xeroderma pigmentosum (XP) fibroblasts. SOD activity in fibroblasts originating from seven xeroderma pigmentosum (XP) patients was significantly lower than that in normal cells (p less than 0.005). Average SOD activity in XP cells belonging to complementation group A was 3.68 +/- 0.54 (n = 7) and that in normal human cells was 5.79 +/- 1.59 (n = 6). Addition of SOD before and during UV irradiation (UVB and UVC) to the cells caused no change in the amount of unscheduled DNA synthesis and UV survival. A possible involvement of reduced SOD in XP and a possible protective effect by SOD on UV damage is discussed.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Cell Survival / radiation effects
  • Cells, Cultured
  • Child
  • Child, Preschool
  • DNA / biosynthesis
  • Female
  • Fibroblasts / enzymology
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Superoxide Dismutase / metabolism*
  • Ultraviolet Rays
  • Xeroderma Pigmentosum / enzymology*
  • Xeroderma Pigmentosum / pathology

Substances

  • DNA
  • Superoxide Dismutase