TDP-43 (a highly conserved nuclear RNA/DNA-binding protein involved in the regulation of RNA processing [171]) is involved in the pathology of familial and sporadic ALS, and distinct TDP-43-mediated ALS models, including Drosophila melanogaster and zebrafish, exhibit defective neuromuscular junctions, dysfunction of locomotor function, and motor neuron defects. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.