Interestingly, neurodegeneration with ataxic features has been associated with defects in factors involved in signaling of DNA DSB damage, for instance ataxia-teleangiectasia (A-T) syndrome (OMIM #208900), a complex condition characterized by ataxia, teleangiectasiae, immunologic deficit, and predisposition to malignancy, caused by ATM mutations; or ataxia-telangiectasia-like syndrome (OMIM #604391), a phenocopy of, but milder than, A-T syndrome, caused by MRE11A mutations. The gene discussed is ATM; the disease is Ataxia.