TARDBP and amyotrophic lateral sclerosis: Although it is not a typical member of the spliceosomal proteins based on the currently available knowledge (Hegele et al., 2012; Korneta et al., 2012), proteopathy of TDP-43 was discovered in the central nervous system that includes hippocampus, neocortex, and spinal cord in patients with frontotemporal lobar degeneration (FTLD-U) or amyotrophic lateral sclerosis (ALS; Neumann et al., 2006; Maekawa et al., 2009).