The most commonly used animal model of ALS is the mutant (MT) human SOD1 (G93A) mouse model.12 Additional mouse models of ALS include those based on other SOD1 mutations,13,14 as well as mutations in TDP-43,15–17FUS/TLS,18,19 and C9ORF72. 20–22SOD1 mouse models have been used in more than 1300 ALS studies,23 with over 60 different molecules yielding protective effects. Here, TARDBP is linked to amyotrophic lateral sclerosis.