Linking DNA damage and neurodegeneration

Trends Neurosci. 2000 Sep;23(9):417-24. doi: 10.1016/s0166-2236(00)01625-8.


Many human pathological conditions with genetic defects in DNA damage responses are also characterized by neurological deficits. These neurological deficits can manifest themselves during many stages of development, suggesting an important role for DNA repair or processing during the development and maintenance of the nervous system. Although the molecular neuropathology associated with such deficits is largely unknown, many of the responsible gene defects have been identified. The current rapid progress in elucidation of molecular details following gene identification should provide further insight into the importance of DNA processing in nervous system function.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.
  • Review

MeSH terms

  • Ataxia Telangiectasia / physiopathology
  • Bloom Syndrome
  • DNA Damage / physiology*
  • DNA Repair
  • Fanconi Anemia / physiopathology
  • Heredodegenerative Disorders, Nervous System / genetics*
  • Heredodegenerative Disorders, Nervous System / physiopathology*
  • Humans
  • Nerve Degeneration / genetics*
  • Nerve Degeneration / physiopathology*
  • Rothmund-Thomson Syndrome / physiopathology
  • Werner Syndrome / physiopathology
  • Xeroderma Pigmentosum / physiopathology