TARDBP and frontotemporal dementia: Neuronal cytoplasmic aggregates containing the nuclear TAR DNA-binding protein 43 (TDP-43) are characteristically observed in the cortical neurons of frontotemporal lobar degeneration (FTLD) with TDP-43 pathology (FTLD-TDP), and in the lower motor neurons of cases with amyotrophic lateral sclerosis (ALS).