Ataxia-telangiectasia (A-T) is a neurodegenerative disease with autosomal recessive inheritance and, despite being monogenic, is characterized by a very pleiotropic phenotype.Since the major functions of ATM are the regulatation of the cellular redox homeostasis and the DNA damage response, lacking of the kinase leads to the impairment of a very large network of signalling pathways, including metabolic pathways.