In most cases of Amyotrophic Lateral Sclerosis (ALS) and Frontotemporal Lobar Degeneration (FTLD), TDP-43 is found to mislocalize from the nucleus to the cytoplasm of affected neurons where it forms ubiquitinated aggregates (Arai et al., 2006; Neumann et al., 2006). The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.