TARDBP and amyotrophic lateral sclerosis: Overall, this model has been proven to fully recapitulate TDP-43-mediated ALS pathology, exhibiting significant similarities with mice models (Gendron and Petrucelli, 2011) and patients, and over the subsequent years it has been used to further investigate different aspects of the disease, in particular TDP-43-regulated genes and their roles in the pathogenesis and pathophysiology of ALS (Godena et al., 2011; Miskiewicz et al., 2014; Langellotti et al., 2018; Romano et al., 2020; Strah et al., 2020; Romano et al., 2021).