Human TDP-43 overexpression also induces cell death in yeast and Drosophila [62], and a high-throughput genetic screening for modifiers of TDP-43-driven gain-of-toxicity in these models resulted in the identification of ataxin-2, one of the strongest risk genes for ALS in humans [62, 63]. This evidence concerns the gene ATXN2 and amyotrophic lateral sclerosis.