Abnormalities in TDP-43, such as cytoplasmic mislocalization and aggregation, are strongly implicated in the pathogenesis of several neurodegenerative disorders, in particular amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD), which are collectively referred to as TDP-43 proteinopathies [3–6]. Here, TARDBP is linked to proteostasis deficiencies.