TDP-43 has been found in the EV fraction of transiently transfected HEK-293 cells (Feiler et al., 2015), as well as EVs isolated from CSF and brains of patients with TDP-43 proteinopathies (Feneberg et al., 2014; Ding et al., 2015; Iguchi et al., 2016). Here, TARDBP is linked to proteostasis deficiencies.