TARDBP and amyotrophic lateral sclerosis: Interestingly, one of the mouse models generated to investigate the underlying causes of TDP-43 mediated neurodegeneration, the prpTDP-43A315T mice, recapitulated many aspects of the human pathology [13], and the cellular events that contribute to CSMN degeneration were identical to the cellular events that are responsible for Betz cell vulnerability and degeneration in ALS patients with TDP-43 pathology [14].