Furthermore, most of ALS-causing mutations, cytotoxic truncated forms, and phosphorylation sites have been associated with the CTD of TDP-43 (Figure 1B) (Hasegawa et al., 2008; Pesiridis et al., 2009; Zhang et al., 2009; Xiao et al., 2015; Berning and Walker, 2019). This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.