Progeroid syndromes and UV-induced oxidative DNA damage

J Investig Dermatol Symp Proc. 2009 Aug;14(1):8-14. doi: 10.1038/jidsymp.2009.6.

Abstract

Progeroid syndromes are a group of diseases characterized by signs of premature aging. These syndromes comprise diseases such as Werner syndrome, Bloom syndrome, Rothmund-Thomson syndrome, Hutchinson-Gilford syndrome, Fanconi anemia, and ataxia-telangiectasia, as well as xeroderma pigmentosum, trichothiodystrophy, and Cockayne syndrome. Clinical symptoms of premature aging are skin atrophy with loss of cutaneous elasticity, dysfunction of cutaneous appendices, degeneration of the central nervous system and an increased susceptibility for malignant tumors. Genetic defects in the repair of DNA damage can lead to progeroid syndromes, and it is becoming increasingly evident that direct DNA damage and indirect damage by highly reactive oxygen species play central roles in aging. The clinical signs of progeroid syndromes and the molecular aspects of UV (ultraviolet radiation)-induced oxidative stress in aging are discussed.Journal of Investigative Dermatology Symposium Proceedings (2009) 14, 8-14; doi:10.1038/jidsymp.2009.6.

Publication types

  • Review

MeSH terms

  • Ataxia Telangiectasia / etiology
  • Bloom Syndrome / etiology
  • Cockayne Syndrome / etiology*
  • Cockayne Syndrome / genetics
  • Cockayne Syndrome / metabolism
  • DNA Damage*
  • DNA Repair
  • Fanconi Anemia / etiology
  • Female
  • Humans
  • Male
  • Models, Biological
  • Oxidative Stress / radiation effects
  • Progeria / etiology
  • Rothmund-Thomson Syndrome / etiology
  • Trichothiodystrophy Syndromes / etiology
  • Ultraviolet Rays / adverse effects*
  • Werner Syndrome / etiology
  • Xeroderma Pigmentosum / etiology