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Neurodegenerative Tauopathies
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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“Corticobasal degeneration (CBD), progressive supranuclear palsy (PSP) and a subset of frontotemporal dementia (FTD) are neurodegenerative disorders characterized by tau inclusions in neurons and glia (tauopathies).”
From Shared genetic risk between corticobasal degeneration, progressive supranuclear palsy, and frontotemporal dementia (2017) · cites this paper as [26]
In the citing paper
“Neurodegenerative tauopathies are characterized by progressive neuronal and glial accumulation in amyloid assemblies of tau protein (Lee et al., 2001).”
From Spontaneous pathology in PS19 tauopathy mice progresses via brain networks (2025) · cites this paper as Lee et al., 2001
In the citing paper
“In the absence of amyloid-beta, NFTs are also implicated in several other neurodegenerative diseases (frontotemporal dementia with parkinsonism, progressive supranuclear palsey, corticobasal degeneration), further supporting the role of tau in the dementing process (Lee et al., 2001).”
From In vivo imaging of tau pathology using multi-parametric quantitative MRI (2015) · cites this paper as Lee et al., 2001
In the citing paper
“Tauopathies include several neurodegenerative diseases such as Pick's disease and frontotemporal dementia with parkinsonism linked to chromosome 17 (FTDP-17; reviewed in 1).”
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Self-citation
Metadata
- Authors
- Lee, V. M.-Y., Goedert, M., & Trojanowski, J. Q.
- Affiliations
- MRC Laboratory of Molecular Biology (GB)
- 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
- 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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