To evaluate the efficacy of ATH-1105 or riluzole in a TDP-43-driven model of ALS, 1-month-old male Prp-TDP43A315T transgenic mice (“ALS mice”) were treated with either ATH-1105 (20 mg/kg, p.o.), riluzole (5 mg/kg, i.p.), or the combination of the two compounds once daily for 2 months. Here, TARDBP is linked to amyotrophic lateral sclerosis.