TARDBP and proteostasis deficiencies: After TDP was identified as the main disease protein in the majority of FTD cases [49], the ubiquitinated compact and skeinlike inclusions characteristic for ALS were also found to be composed of TDP-43 [49, 50], thereby providing strong evidence that ALS and FTD are part of a clinicopathological continuum of multisystem diseases, the so-called TDP-43 proteinopathies [7].