Dominant mutations in the gene encoding TDP-43 (TARDBP) that cause mislocalization of the protein were subsequently identified in cohorts of patients with ALS and FTLD (Gitcho et al., 2008; Kabashi et al., 2008; Sreedharan et al., 2008; Van Deerlin et al., 2008; Yokoseki et al., 2008). This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.