Loss of TDP-43 specifically in motor neurons results in cell death and an ALS-like phenotype in mice (Wu et al., 2012) and reduced TDP-43 expression in Drosophila and zebrafish results in motor deficits (Feiguin et al., 2009; Kabashi et al., 2010). This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.