ALS is an adult-onset motor neuron disease caused by mutations in the genes encoding Cu/Zn superoxide dismutase (SOD1), alsin, senataxin, (FUS), vesicle-associated membrane protein-associated protein B (VAPB), TAR DNA-binding protein (TARDBP), and dynactin 1 (DCTN1) [3]. Here, SETX is linked to amyotrophic lateral sclerosis.