Given that TDP-43 proteinopathy primarily affects the MN in ALS and FTD brains, we exploited the MN-Tdp-43∆NLS mouse line for histopathological and biochemical analyses relevant to ALS/FTD-TDP-43 pathology in human patients, unless stated otherwise in the following results. The gene discussed is TARDBP; the disease is proteostasis deficiencies.