TDP-43 is the major disease-associated protein involved in the pathogenesis of amyotrophic lateral sclerosis (ALS), frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTLD) linked to TDP-43 pathology (FTLD-TDP)14, 15, and other neurodegenerative disorders16, 17, 18, 19, 20. Here, TARDBP is linked to frontotemporal dementia.