The TDP-43 proteinopathies may also result in the impairment of nucleocytoplasmic transport (NCT) [132,133,134] and axonal transport [81,135], induction of the endoplasmic reticulum and oxidative stresses [136,137,138], apoptosis [139,140], dysfunction of mitochondrion [141,142,143] and also deregulation of protein quality control system (such as UPS and autophagy) [144,145]. The gene discussed is TARDBP; the disease is proteostasis deficiencies.