Similar to TDP-43 pathology, FUS-related FTLD/ALS pathology is characterized by mislocalization of FUS to the cytoplasm and a concomitant reduction in nuclear expression in affected neurons (Neumann et al., 2009; Deng et al., 2010; Mackenzie et al., 2011). The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.