TARDBP and amyotrophic lateral sclerosis: The presence of ubiquitinated and phosphorylated cytoplasmic inclusions of TDP-43 in the CNS of patients with ALS and FTLD [119], and the subsequent identification of TARDBP mutations in the sporadic and familial forms of these diseases [57, 79, 160, 175, 192], have led to the rapid development of TDP-43 transgenic models.