DNA damage as an upstream pathogenic event in human ALS is supported by p53 activation and its import into the nucleus of motor neurons [64], widespread brain activation of poly (ADP-ribose) polymerase [48], and hyperactivation and nuclear accumulation of apurinic/apyrimidinic endodeoxyribonuclease-1 [111]. Here, TP53 is linked to amyotrophic lateral sclerosis.