TDP-43 is a protein first discovered in 2006 to be present within the ubiquitinated inclusions that are a hallmark of amyotrophic lateral sclerosis (ALS) and in many cases of Tau-negative frontotemporal lobar degeneration (FTLD-TDP; Neumann et al., 2006; Cairns et al., 2007). The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.