TARDBP and proteostasis deficiencies: Several other studies demonstrated the potential contribution of TDP-43 deficiency to disease pathogenesis [10], [12], while our group and others have demonstrated that overexpression of the human TDP-43 (hTDP-43) protein, either wild-type (hTDP-43WT) or mutant hTDP-43, leads to pathological phenotypes consistent with certain TDP-43 proteinopathies.