Early studies of ataxia-telangiectasia, a childhood disease affecting the development of the neuron system caused by mutations in ATM, a serine/threonine kinase that plays a critical role in repairing DNA double-strand breaks (DSBs), provided the first linkage between DNA damage and neurodegeneration(Savitsky et al., 1995; Rothblum-Oviatt et al., 2016). The gene discussed is ATM; the disease is ataxia telangiectasia.