DNA damage and DNA repair deficiency have long been associated with neurodegeneration as mutations in DDR and DNA repair genes have been found to cause a number of neurodegenerative diseases, including ataxia telangiectasia, ataxia telangiectasia-like disorder, Nijmegen breakage syndrome, ataxia-oculomotor apraxia-1 and 2, ALS4 (caused by mutations in SETX, [27]), spinocerebellar ataxia with axonal neuropathy-1, and Cockayne syndrome [42]. Here, SETX is linked to ataxia telangiectasia.