The formation of β-sheet-rich neurofibrillary tangles (NFT) caused by the abnormal aggregation of misfolded microtubule-associated protein Tau is a hallmark of tauopathies such as Alzheimer’s Disease (AD), frontotemporal dementia with Parkinsonism (FTDP-17), Progressive Supranuclear Palsy (PSP), Pick’s disease (PD) and others. Here, MAPT is linked to early-onset autosomal dominant Alzheimer disease.