TARDBP and proteostasis deficiencies: Although TDP-43 proteinopathies have been identified in a wide range of neurodegenerative diseases including sporadic ALS, FTLD, Alzheimer's disease, and dementia with Lewy bodies [5]–[9], TDP-43 inclusions have not been detected in familial ALS caused by mutation of the SOD1 and FUS genes [10]–[13].