Since the identification of a nuclear protein, 43-kDa TAR DNA-binding protein (TDP-43, also known as TARDBP), as the major component of the ubiquitinated inclusions in frontotemporal lobar degeneration with ubiquitin-positive, tau-and α-synuclein-negative inclusions (FTLD-U) and ALS [11, 12], many studies of such cases employing TDP-43 immunohistochemistry have been performed [13–18]. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.