The discovery of the TAR DNA binding protein 43 (TDP-43) as a component of the neuronal inclusions in most cases of amyotrophic lateral sclerosis (ALS), and many cases of frontotemporal lobar degeneration (FTLD) marked a significant advance in the research into these devastating neurodegenerative diseases [1, 2]. The gene discussed is TARDBP; the disease is frontotemporal dementia.