TARDBP and proteostasis deficiencies: These mice express either nuclear (TDP-43-WT) or cytoplasmic (TDP-43-ΔNLS) forms of the protein, and they recapitulated several aspects of TDP-43 proteinopathies, including time-dependent neuronal loss, gliosis, corticospinal tract degeneration and global changes in gene expression (Igaz et al., 2011).