Given the strong in vitro evidence that HEXA-018 attenuates TDP-43-induced toxicity in neuronal cells, we next examined whether HEXA-018 treatment suppresses TDP-43-induced toxicity in a Drosophila model of TDP-43 proteinopathy that expresses human TDP-43 in the nervous system (Kim et al., 2014; Lee et al., 2020a). This evidence concerns the gene TARDBP and proteostasis deficiencies.