Mislocalized tau proteins abnormally aggregate into intracellular, filamentous inclusions (neurofibrillary tangles; NFTs) in brains of individuals with neurodegenerative diseases including Alzheimer’s disease (AD), progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), argyrophilic grain disease (AGD), Pick’s disease, and familial frontotemporal lobar degeneration with underlying tau pathology (FTLD-Tau), all collectively referred to as tauopathies (Lee et al., 2001). The gene discussed is MAPT; the disease is early-onset autosomal dominant Alzheimer disease.