TARDBP and frontotemporal dementia: Furthermore, at the endpoint of >95% of ALS cases and ∼45% of FTD cases, the RNA/DNA-binding protein TDP-43 (TAR DNA-binding protein of 43 kDa) abnormally accumulates in ubiquitin-positive inclusions in the cytoplasm of affected neurons and glia (Arai et al., 2006; Mackenzie et al., 2007; Neumann et al., 2006).