TAR DNA binding protein 43 (TDP-43) was identified as the major ubiquitinated protein, with positive inclusions in neurons and glia, occurring in FTD and approximately half of FTD patients have TDP 43 inclusions due to mutations in the progranulin gene found in the frontal and temporal neurons and dentate gyrus. The gene discussed is TARDBP; the disease is frontotemporal dementia.