There is a pressing demand for FTLD-TDP-specific biomarkers (55), and ELISAs capable of accurately measuring TDP-43 levels in CSF and/or plasma are crucial not only for diagnosis but also for monitoring responses to disease-modifying therapies in FTLD-TDP and other TDP-43 proteinopathies (56). The gene discussed is TARDBP; the disease is proteostasis deficiencies.