In addition to its occurrence in ALS, the cytoplasmic accumulation of TDP-43 in neurons is also observed in frontotemporal dementia (FTD; about 50% of patients) (Neumann et al., 2006), as well as in Alzheimer’s (Amador-Ortiz et al., 2007), Parkinson’s (Nakashima-Yasuda et al., 2007), and Huntington’s diseases (Schwab et al., 2008), although with lower frequency compared to ALS. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.