Neuropathological analysis of human brain tissue showed that acetylated tau at residue K280 (Lys280) within the tau MTBR accumulated in tauopathy brains and produced a distinctly pathological signature in Alzheimer’s disease (AD) and also a panel of other tauopathies including corticobasal degeneration (CBD), progressive supranuclear palsy (PSP), and several FTDP-17 familial dementia patients. The gene discussed is MAPT; the disease is early-onset autosomal dominant Alzheimer disease.