Almost all fALS cases are due to an autosomal dominant inheritance, however, only 50% of them can be associated with specific ALS-related genes, which mainly consist of C9ORF72 (40%), SOD1 (20%), FUS (1–5%), and TARBDP (1–5%, encoding for the canonical 43-kDa transactive response DNA binding protein, TDP-43) [4]. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.