Several different rat and mouse models, in which wild-type or mutant TARDBP or mutant FUS bearing ALS-associated mutations are overexpressed, develop cytoplasmic aggregation and exhibit features of ALS and FTD, including cortical and hippocampal neuronal loss and motor deficits (Huang et al., 2011; Igaz et al., 2011; Scekic-Zahirovic et al., 2016; Sharma et al., 2016; Tsai et al., 2010; Wils et al., 2010; Xu et al., 2010). This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.