Approximately 50% of cases with frontotemporal lobar degeneration (FTLD), the neuropathological diagnosis of FTD, are characterized by cytoplasmic inclusions containing TAR DNA binding protein 43 (TDP-43) in neurons and glia, with a concomitant loss of nuclear TDP-43 [3, 4]. The gene discussed is TARDBP; the disease is frontotemporal dementia.