The goals of the current work were three-fold: (1) to expand on our understanding of the therapeutic potential of ATH-1105 in a TDP-43 driven mouse model of ALS by comparing and combining it with the known efficacious treatment riluzole, (2) to understand the effect of ATH-1105 on some of the cellular mechanisms associated with TDP-43 pathology, and (3) to verify that the mechanism of action of ATH-1105 is dependent on MET signaling in motor neurons, as evidence of target engagement in vitro. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.