TARDBP and frontotemporal dementia: Transactive response DNA-Binding Protein 43 (TDP-43, 43 kDa) has been characterized as a major component of cytoplasmic protein inclusion and surprisingly detectable in approximately 97% of patients with sporadic ALS (sALS) and familial ALS (fALS) or approximately 50–55% of frontotemporal dementia patients, indicating the possible link of TDP-43 dysfunction to neurodegeneration1.