Thus far, more than 10 ALS-associated loci have been assigned, and several causative genes, including SOD1 (ALS1), ALS2, SETX (ALS4), SPATACSIN/SPG11 (ALS5), FUS (ALS6), VAPB (ALS8), ANG (ALS9), and TARDBP (ALS10) have been identified and characterized [1], [2], [3]. Here, SETX is linked to amyotrophic lateral sclerosis.