Cited by 2,480Alzheimer's disease research and treatmentsDementia and Cognitive Impairment ResearchAmyotrophic Lateral Sclerosis Research

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Neurodegenerative Tauopathies

Lee, Virginia M-Y, Goedert, Michel, Trojanowski, John Q·2001·Annual Review of Neuroscience

DOI 10.1146/annurev.neuro.24.1.1121

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Abstract

The defining neuropathological characteristics of Alzheimer's disease are abundant filamentous tau lesions and deposits of fibrillar amyloid beta peptides. Prominent filamentous tau inclusions and brain degeneration in the absence of beta-amyloid deposits are also hallmarks of neurodegenerative tauopathies exemplified by sporadic corticobasal degeneration, progressive supranuclear palsy, and Pick's disease, as well as by hereditary frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17). Because multiple tau gene mutations are pathogenic for FTDP-17 and tau polymorphisms appear to be genetic risk factors for sporadic progressive supranuclear palsy and corticobasal degeneration, tau abnormalities are linked directly to the etiology and pathogenesis of neurodegenerative disease. Indeed, emerging data support the hypothesis that different tau gene mutations are pathogenic because they impair tau functions, promote tau fibrillization, or perturb tau gene splicing, thereby leading to formation of biochemically and structurally distinct aggregates of tau. Nonetheless, different members of the same kindred often exhibit diverse FTDP-17 syndromes, which suggests that additional genetic or epigenetic factors influence the phenotypic manifestations of neurodegenerative tauopathies. Although these and other hypothetical mechanisms of neurodegenerative tauopathies remain to be tested and validated, transgenic models are increasingly available for this purpose, and they…

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Authors: Lee, V. M.-Y., Goedert, M., & Trojanowski, J. Q.
Affiliations: MRC Laboratory of Molecular Biology (GB)
ORCID: Lee, Virginia M-Y
Goedert, Michel
Trojanowski, John Q
Venue: Annual Review of Neuroscience
Publisher: Annual Reviews
Published: 2001-03
Volume: 24
Issue: 1
Pages: 1121-1159
ISSN: 0147-006X (ISSN-L)
1545-4126
DOI: 10.1146/annurev.neuro.24.1.1121
OpenAlex: W4230565756
Semantic Scholar: 8eb6355272daf41b8da72a5b18a4d8c67d8509cc
Cited by: 2,480
References: 245
Citations by year: 2026: 60 · 2025: 81 · 2024: 102 · 2023: 112 · 2022: 133 · 2021: 130 · 2020: 126 · 2019: 107 · 2018: 114 · 2017: 94 · 2016: 100 · 2015: 115 · 2014: 99 · 2013: 100 · 2012: 92
FWCI: 9.22
Type: journal article
Language: en
Open access: closed
Locations: Annual Review of Neuroscience (publishedVersion)
PubMed (publishedVersion)
Topics: Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Alzheimer's disease research and treatments
Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
Health Sciences › Medicine › Clinical Neurology › Amyotrophic Lateral Sclerosis Research
Keywords: tau protein, tau gene mutations, FTDP-17, progressive supranuclear palsy, corticobasal degeneration, Pick's disease, Alzheimer's disease, tau fibrillization, genetic risk factors, transgenic animal models, neurodegeneration
MeSH terms: Animals, Brain, Brain Diseases, Dementia, Humans, Neurofibrillary Tangles, tau Proteins

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