These TDP-43 aggregates have been documented in almost all cases of sALS and fALS, except for SOD1-linked fALS, and are characterized by the invariable presence of phosphorylated forms of full-length (FL) and fragmented TDP-43; thus TDP-43 aggregation is considered as a pathological signature for ALS (3,7). This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.