TARDBP and proteostasis deficiencies: ALS and FTD are also linked to TDP-43 proteinopathy, which is present in both familial and sporadic cases, where cytoplasmic aggregates of abnormally phosphorylated and ubiquitinated TDP-43 are present in neurons and glial cells of various CNS areas, thus causing neurodegeneration due to both toxicity and loss of function of mutant TDP-43 [107].