TDP-43 proteinopathy characterized by the cytoplasmic aggregation and nuclear depletion of TDP-43 is a common pathological feature of several neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS), frontotemporal lobar degeneration (FTLD), and Alzheimer’s disease (AD) (1, 4, 5). Here, TARDBP is linked to frontotemporal dementia.