TARDBP and proteostasis deficiencies: Although it does not fully recapitulate the pathological features of TDP-43 proteinopathies, artificial axonal damage induces transient cytoplasmic distribution of TDP-43 in mouse motor neurons [9], [10], and several stress conditions, including oxidative stress and suppression of the ubiquitin-proteasome system, cause aberrant modifications of TDP-43 in cultured cell lines or primary neurons [11]–[15].