PMID- 11898551 OWN - NLM STAT- MEDLINE DCOM- 20020409 LR - 20191105 IS - 1528-4042 (Print) IS - 1528-4042 (Linking) VI - 1 IP - 5 DP - 2001 Sep TI - Frontotemporal dementia and tauopathy. PG - 413-21 AB - The presence of abundant neurofibrillary lesions made of hyperphosphorylated tau proteins is the characteristic neuropathology of a subset of neurodegenerative disorders classified as "tauopathies." The discovery of mutations in the tau gene in frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17) constitutes convincing evidence that tau proteins play a key role in the pathogenesis of neurodegenerative disorders. Moreover, it now is known that the most common form of sporadic frontotemporal dementia (FTD), which is characterized by frontotemporal neuron loss, gliosis, and microvacuolar change, also is a tauopathy caused by a loss of tau protein expression. Thus, these discoveries have begun to change the classification and the neuropathologic diagnosis of FTD and tauopathies, as well as current understanding of the disease mechanisms underlying them. Although transgenic mice expressing wild-type human tau or variants thereof with an FTDP-17 mutation result in tau pathologies and brain degeneration similar to that seen in human tauopathies, the precise mechanisms leading to the onset and progression of neurodegenerative disorders remain incompletely understood. Here, we review current understanding of human neurodegenerative tauopathies and prospects for translative recent insights about these into therapeutic interventions to prevent or ameliorate them. FAU - Yoshiyama, Y AU - Yoshiyama Y AD - Center for Neurodegenerative Research, Department of Pathology and Laboratory Medicine, University of Pennsylvania, 3400 Spruce Street, 3rd Floor Maloney, Philadelphia, PA 19104, USA. FAU - Lee, V M AU - Lee VM FAU - Trojanowski, J Q AU - Trojanowski JQ LA - eng PT - Journal Article PT - Review PL - United States TA - Curr Neurol Neurosci Rep JT - Current neurology and neuroscience reports JID - 100931790 RN - 0 (tau Proteins) SB - IM MH - Dementia/*genetics/*pathology MH - Humans MH - Tauopathies/genetics/pathology MH - tau Proteins/genetics RF - 50 EDAT- 2002/03/20 10:00 MHDA- 2002/04/10 10:01 CRDT- 2002/03/20 10:00 PHST- 2002/03/20 10:00 [pubmed] PHST- 2002/04/10 10:01 [medline] PHST- 2002/03/20 10:00 [entrez] AID - 10.1007/s11910-001-0100-0 [doi] PST - ppublish SO - Curr Neurol Neurosci Rep. 2001 Sep;1(5):413-21. doi: 10.1007/s11910-001-0100-0.