Recently, Tar DNA-binding protein (TARDBP) gene-encoded product TDP-43 (TDP) was discovered as the major component of the signature inclusion bodies in ALS/FTLD-U [3], [4], and several other neurodegenerative diseases, collectively called TDP proteinopathy [5], [6]. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.