TDP-43 is heavily implicated as a key causal factor in the neurodegenerative diseases (amyotrophic lateral sclerosis [ALS]) and frontotemporal dementia (FTD), both of which show some degree of area-selective pathology in the neocortex of both patients and animal models (Taylor et al., 2016). The gene discussed is TARDBP; the disease is frontotemporal dementia.