Formation of TAR-DNA binding protein (TARDBP, also known as TDP43)-positive intracellular inclusions in the central nervous system (CNS) is the most common pathology in the patients with ALS and frontotemporal lobar degeneration (FTD), even in the absence of TDP-43 mutations [31-33]. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.