The umbrella term “TDP-43 proteinopathies” was coined shortly after the discovery that most forms of ALS and around 45% of FTD cases have TDP-43-positive neuronal and glial inclusions as a major pathological hallmark (Arai et al., 2006; Neumann et al., 2006; Cairns et al., 2007). The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.