Ubiquitinated, phosphorylated TDP-43 wild type or mutant protein and carboxyl-terminal degradation products constitute major components of intranuclear and cytoplasmic neuronal inclusions that are observed in the majority of ALS variants except for those caused by SOD1 mutations, highlighting alteration of mRNA-processing [137] and mRNA-binding [138] as one of the critical pathophysiological disease mechanisms [10]. Here, TARDBP is linked to amyotrophic lateral sclerosis.