In 2006, TAR DNA-binding protein of 43 kDa (TDP-43) was identified as a major component of the ubiquitin-positive inclusions that accumulate in the brains of patients with amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD), and the diseases in which abnormal TDP-43 accumulates are collectively called TDP-43 proteinopathies [14, 216]. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.