Mouse/Rat—represents some features of clinical ALS, wide range of phenotype variation (some lines no MN phenotype), no clear aggregation or loss of nuclear TDP-43 (Chen-Plotkin et al., 2010; Tsao et al., 2012) Zebrafish—recapitulates some hallmarks of ALS, strong MN degeneration, cytoplasmic TDP-43 Fly—recapitulates some ALS aspects, strong MN degeneration documented, cytoplasmic TDP-43 (Casci and Pandey, 2015) Worm—MN degeneration phenotype (Therrien and Parker, 2014). This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.