Remarkably, although TARDBP mutations account for only 5–10% of familial ALS and the remaining over 90% are attributable to other genes such as C9ORF72, SOD1, FUS, and UBQLN2 (Kim et al., 2020), the majority of ALS cases (about 97%) also exhibit TDP-43-induced pathology (Ling et al., 2013). This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.