TAR DNA-binding protein 43 (TDP-43) encoded by TARDBP, a DNA/RNA-binding protein that localizes predominantly to the nucleus and shuttles between the cytoplasm and nucleus [5], is detected as a main constituent of cytoplasmic aggregates in neuronal and glial cells in patients with ALS and FTLD with TDP-43-positive inclusions (FTLD-TDP) [6,7]. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.