Hyperphosphorylated TDP-43 aggregates are considered a hallmark of ALS-FTD, in particular, the phosphorylated residues Ser-379, Ser-403, Ser-404, and Ser-409/Ser-410 are the most recurrent on ALS-FTD TDP-43 inclusions [249,310]. Here, TARDBP is linked to amyotrophic lateral sclerosis.