FUS (1-359) mice express a version of the FUS protein that lacks its entire NLS and RNA-binding motifs, but they share several phenotypic hallmarks with ALS-FUS patients, who predominantly present an aggressive clinical course, severe neuronal loss in the spinal cord ventral horn (Ravits et al., 2013), and mislocalisation and aggregation of FUS protein in the cytoplasm (Shelkovnikova et al., 2013; Hewitt et al., 2010). This evidence concerns the gene FUS and amyotrophic lateral sclerosis.