Although TDP-43 is an RNA/DNA binding protein that physiologically resides in the nucleus, hyperphosphorylated extranuclear inclusions are found in neuronal cells of approximately 45% of patients with FTD and approximately 97% of patients with ALS (Ling et al., 2013; Neumann et al., 2006). This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.