The cytoplasmic inclusion composing of TDP-43 and FUS in motor and/or cortical neurons is observed in FTLD/ALS as a major pathological hallmark, as observed in other neurodegenerative disorders (Neumann et al., 2006, 2009; Mackenzie and Neumann, 2012). The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.