TAR DNA-binding protein 43 (TDP-43) is a protein constituent of pathologic cytoplasmic and intranuclear inclusions in neurons and glia of patients with sporadic and familial forms of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) [1], [2]. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.