In an FTLD-U mouse model that exhibits TDP-43 proteinopathy due to overexpression of wild-type TDP-43, treatment with various autophagy activators (rapamycin, spermidine, carbamepine and tamoxifen) rescued motor dysfunction, reducing the level of TDP-43 inclusions and CTFs generated and alleviating caspase-mediated apoptosis [190]. This evidence concerns the gene TARDBP and proteostasis deficiencies.