Mutations in TARDBP account for 3–5% of cases of familial ALS and less than 1% of idiopathic ALS.10,11 More than 80 ALS-linked dominant mutations in TARDBP have been described in patients with ALS, but the specific pathomechanisms of these TARDBP variants remain unclear. Here, TARDBP is linked to amyotrophic lateral sclerosis.