TARDBP and proteostasis deficiencies: The common features of TDP-43 proteinopathies are an accumulation of TDP-43 in insoluble, cytoplasmic inclusions, concomitant with a loss of nuclear TDP-43, the presence of truncated 20–25 kDa and 35 TDP-43 C-terminal fragments, and abnormal phosphorylation and ubiquitination of TDP-43 in the upper and lower motor neurons and in other regions of the central nervous system (Geser et al. 2010; Mackenzie et al. 2010).