"Frontotemporal Lobar Degeneration (FTLD) Biomarker Assays"Our discovery that TDP-43 is the disease protein in FTLD and amyotrophic lateral sclerosis (ALS) is the impetus for this grant. Although FTLD and ALS appear to be clinically distinct disorders, both show nervous system accumulations of similar inclusions that were of unknown composition until 2006 when we discovered that TDP-43 forms the inclusions of both ALS and FTLD. This year, mutations in the TDP-43 gene (TARDBP) were shown to cause FTLD with ALS. Hence, the goals of this grant are translate these exciting new discoveries into TDP-43 blood and spinal fluid tests for the early diagnosis of FTLD in order to expedite clinical trials of disease modifying therapies and to make these diagnostic assays available to the FTLD and neurodegenerative disease research community.