To date, four major proteinopathies have been identified in FTD patients: Tau, TAR DNA-binding protein 43 (TDP-43), fused in sarcoma (FUS), and ubiquitin positive inclusions, the latter comprising unknown aggregated proteins; with the first two accounting for the vast majority of cases (Neumann et al., 2009; MacKenzie et al., 2010). Here, FUS is linked to proteostasis deficiencies.