TARDBP and frontotemporal dementia: The 43 kDa TAR DNA-binding protein (TDP-43) and its C-terminal proteolytic fragments have been identified as the major component of inclusion bodies in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with unbiquitin-positive inclusions (FTLD-U) [1], [2], [3], [4], [5].