In the Center for Neurodegenerative Disease Research (CNDR) brain bank, TDP-43 accumulates as a major disease causing proteinopathy in 88% of amyotrophic lateral sclerosis (ALS) patients, 55% of late-onset Alzheimer’s disease (AD) patients where it is described as limbic-predominant age-related TDP-43 encephalopathy neuropathologic change (LATE-NC), and 42% of frontotemporal degeneration (FTD) patients where it is known as frontotemporal lobar degeneration with TDP-43 inclusions (FTLD–TDP). Here, TARDBP is linked to early-onset autosomal dominant Alzheimer disease.