In this Review, familial and sporadic neurological disorders reported to have an etiological link with DNA repair defects are ... and four (ataxia-telangiectasia, ataxia-telangiectasia-like ...

DNA integrity is always under ... These disorders include ataxia-telangiectasia (AT), a degenerative motor condition caused by failure to repair oxidative damage in the cerebellum, and xeroderma ...

Defects in homologous ... and/or ATR (ataxia telangiectasia and Rad3-related) kinases, which mediate cell cycle checkpoint control; and the sensing and repair of DNA breaks. We review the data ...

A number of these disorders have a definitive link to an inherited or acquired defect in one of the DNA repair pathways. Table 1 lists these disorders along with the known genetic DNA repair ...