FUS and amyotrophic lateral sclerosis: The latter hypothesis is supported by several pieces of evidence, including the fact that TDP-43 or FUS nuclear staining is decreased in the nuclei of neurons in both human FTLD/ALS tissue (Davidson et al., 2007; Neumann et al., 2009) and TDP-43-overexpressing mice (Wegorzewska et al., 2009; Igaz et al., 2011).