The limited clinical efficacy of compounds tested on SOD1 mice (Vucic et al., 2014), as well as the notion that SOD1 mutations account only for a small number (~2%) of sporadic ALS cases (Renton et al., 2013) prompted the generation of new rodent ALS-models, like mice with mutated or overexpressed TDP-43 or fused in sarcoma (FUS) (Da Cruz and Cleveland, 2011; Van Den Bosch, 2011; Wegorzewska and Baloh, 2011; McGoldrick et al., 2013). This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.