Neurons and DNA repair: neurologic involvement in xeroderma pigmentosa.
Involvement of the nervous system occurs in only some forms of xeroderma pigmentosa. A hypothesis is proposed explaining the relative vulnerability of neurons in the presence of impaired DNA repair mechanisms. A large portion of the genome is expressed in the brain, making it more susceptible to the effects of accumulated mutations. In the setting of cells which do not divide, a moderate decrease in the efficiency of the DNA repair systems may manifest in premature cell death.
Grewal
R P
RP
Developmental and Metabolic Neurology Branch, NINDS, National Institute of Health, Bethesda, Maryland 20892.
D004247:DNA / Q000235:genetics; D004260:DNA Repair; D004261:DNA Replication; D006801:Humans; D008954:Models, Biological; D009474:Neurons / Q000502:physiology*; D014983:Xeroderma Pigmentosum / Q000235:genetics / Q000503:physiopathology*
D004247:DNA