NCT dysfunction has been implicated in FUS and TAR-DNA binding protein 43 kDa (TDP-43) proteinopathies because both of these proteins are normally enriched in the nucleus, although in ALS/FTD spectrum disorders, they are mislocalized in the cytoplasm (Arai et al., 2006; Dormann et al., 2010). The gene discussed is TARDBP; the disease is proteostasis deficiencies.