In 2008, dominant mutations in the TARDBP gene were identified as a primary cause of ALS, providing evidence that aberrant TDP-43 could be causative of neurodegeneration (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.