Today, both diseases fall under the umbrella term of TDP-43 proteinopathies, in reference to the occurrence of cytoplasmic inclusions of the TAR DNA-binding protein 43 (TDP-43) in glial and neuronal cells: 97% of ALS as well as almost 50% of FTD cases present with such TDP-43-positive inclusions (Cairns et al., 2007; Mackenzie et al., 2007). The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.