FUS and amyotrophic lateral sclerosis: The FUSDelta14 KI heterozygous mice, expressing a partially humanised mutant FUS gene, carrying a splice acceptor site mutation that results in a frameshift that causes an aggressive form of ALS in humans, show progressive spinal MN loss, cytoplasmic mislocalisation of FUS and impaired lipid metabolism (Devoy et al., 2017) (Table 1C).