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Tau is a microtubule associated protein which promotes polymerization of tubulin and the major component of Alzheimer’s neurofibrillary tangles. Tau is encoded in a single gene located at 17q21-22 and composed of six isoforms in adult human brain by alternative mRNA splicing. Mutations in this gene cause frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17). The mechanism of these diseases can be considered in terms of reduced microtubule polymerization promoting activities or of abnormal expression of tau isoforms. We review here current comprehension of clinicopathological significance of tau and discuss the pathogenesis of these diseases.