Tauopathies.
Abstract
Tauopathies are neurodegenerative disorders characterized by the deposition of abnormal tau protein in the brain. The spectrum of tau pathologies expands beyond the traditionally discussed disease forms like Pick disease, progressive supranuclear palsy, corticobasal degeneration, and argyrophilic grain disease. Emerging entities and pathologies include globular glial tauopathies, primary age-related tauopathy, which includes neurofibrillary tangle dementia, chronic traumatic encephalopathy (CTE), and aging-related tau astrogliopathy. Clinical symptoms include frontotemporal dementia, corticobasal syndrome, Richardson syndrome, parkinsonism, pure akinesia with gait freezing and, rarely, motor neuron symptoms or cerebellar ataxia. Some disorders show specific neuroimaging features, while examination of the cerebrospinal fluid awaits markers for in vivo stratification of cases. The possibility of cell-to-cell propagation is a novel aspect of the pathogenesis of tauopathies, which is partly reflected by the hierarchic involvement of anatomic regions. This concept might have relevance for the development of therapies. For cost-effective screening for tau pathologies in neuropathologic practice, examination of the hippocampus, amygdala, and basal ganglia is recommended. Uncommon morphologies or unusually extensive forms of tau pathologies should raise the suspicion of a genetic background. Ongoing multidisciplinary studies are needed to understand the whole spectrum and significanc…
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Under pathological conditions, hyperphosphorylation of tau reduces its affinity for tubulin and is thought to drive abnormal aggregations of phosphorylated tau, such as neuropil threads or neurofibrillary tangles, resulting in tauopathies.
“However, under pathological conditions, the hyperphosphorylation of tau reduces its affinity for tubulin and is thought to drive abnormal aggregations of phosphorylated tau (p-tau), such as neuropil threads or neurofibrillary tangles (NFTs), resulting in tauopathies (Wang and Mandelkow, 2016; Kovacs, 2017).”
From Tauopathy and Epilepsy Comorbidities and Underlying Mechanisms (2022) · cites this paper as Kovacs, 2017
In classical tauopathies where overt epilepsy infrequently occurs, tau hyperphosphorylation can arise from a variety of different causes.
“In classical tauopathies where overt epilepsy infrequently occurs, tau hyperphosphorylation can arise from a variety of different causes (Kovacs, 2017).”
From Tauopathy and Epilepsy Comorbidities and Underlying Mechanisms (2022) · cites this paper as Kovacs, 2017
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- Authors
- Kovács, G. G.
- Affiliations
- Medical University of Vienna (AT)
- Corresponding author
- Gábor Géza Kovács
- ORCID
- Gábor Géza Kovács
- Venue
- Handbook of Clinical Neurology
- Publisher
- Elsevier
- Published
- 2018
- Volume
- 145
- Pages
- 355-368
- ISSN
- 0072-9752 (ISSN-L)
- OpenAlex
- W4247443907
- Cited by
- 225
- References
- 73
- Citations by year
- 2026: 14 · 2025: 19 · 2024: 41 · 2023: 38 · 2022: 35 · 2021: 36 · 2020: 20 · 2019: 15 · 2018: 6 · 2014: 1
- FWCI
- 36.00
- Type
- book chapter
- Language
- en
- Open access
- closed
- Locations
- Handbook of clinical neurology (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
- Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Keywords
- tauopathy, tau protein, neurodegenerative diseases, Pick's disease, progressive supranuclear palsy, corticobasal degeneration, argyrophilic grain disease, globular glial tauopathy, primary age-related tauopathy, chronic traumatic encephalopathy, frontotemporal dementia, corticobasal syndrome, Richardson syndrome, cell-to-cell transmission, cerebrospinal fluid biomarkers, neuroimaging
- MeSH terms
- Animals, Brain, Humans, tau Proteins, Tauopathies
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