title: Relation of D.N.A. repair processes to pathological ageing of the nervous system in xeroderma pigmentosum.
abstract: The severity of neurological abnormalities in patients with xeroderma pigmentosum has been found to be related to their ability to repair ultraviolet (U.V.)-damaged D.N.A. Patients with the most severe neurological abnormalities have the least effective D.N.A. repair is shown by the decreased colony-forming ability of their U.V.-irradiated fibroblasts. These results suggest that the lack of adequate D.N.A. repair is causally related to the clinical manifestations of a human heredodegenerative nervous system disease.
mesh_terms: D000375:Aging; D002455:Cell Division / Q000528:radiation effects; D002470:Cell Survival; D004260:DNA Repair / Q000528:radiation effects; D005347:Fibroblasts / Q000473:pathology; D006801:Humans; D066298:In Vitro Techniques; D009410:Nerve Degeneration; D009422:Nervous System Diseases / Q000209:etiology / Q000235:genetics* / Q000473:pathology; D009474:Neurons / Q000473:pathology; D011829:Radiation Dosage; D012867:Skin / Q000473:pathology; D014466:Ultraviolet Rays; D014983:Xeroderma Pigmentosum / Q000150:complications / Q000235:genetics* / Q000473:pathology