tauopathy
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Introduction
Disorders characterized by abnormal deposition of the mt-tau:
- Alzheimer's disease
- frontotemporal dementias
- corticobasal degeneration
- progressive supranuclear palsy
- pallidopontonigral degeneration
- dementia pugilistica
- amyotrophic lateral sclerosis-parkinsonism/dementia complex of Guam
- argyrophilic grain disease
- Down syndrome
- diffuse neurofibrillary tangles with calcification
- Gerstmann-Straussler-Scheinker disease
- myotonic dystrophy
- Nonaka distal myopathy
- Niemann-Pick disease type C
- non Guamanian motor neuron disease with neurofibrillary tangles
- subacute sclerosing panencephalitis
- postencephalitic parkinsonism
- prion disease with neurofibrillary tangles
- tangle only dementia
Notes
- tau aggregates vary in phosphorylation & isoform content.
- in Alzheimer's disease, all 6 tau isoforms are phosphorylated & aggregate into paired helical filaments
- in corticobasal degeneration & progressive supranuclear palsy, only the phosphorylated 4R tau isoform (containing 4 microtubule binding domains) aggregates
More general terms
More specific terms
- Alzheimer's disease (AD)
- amyotrophic lateral sclerosis-parkinsonism-dementia complex of Guam
- argyrophilic grain disease (AgD)
- chronic traumatic encephalopathy (CTE)
- cortical-basal ganglionic degeneration; corticobasal degeneration
- dementia pugilistica
- Down's syndrome
- frontotemporal dementia; frontotemporal lobar degeneration; frontotemporal neurocognitive disorder (FTD, FTLD)
- Gerstmann-Straussler-Scheinker disease
- myotonic dystrophy; Steinert disease; myotonia dystrophica
- Niemann-Pick disease type C
- Nonaka distal myopathy
- pallidopontonigral degeneration
- Pick's disease
- post-encephalitic parkinsonism
- progressive supranuclear palsy; Steele-Richardson-Olszewski syndrome (PSP)
- subacute sclerosing panencephalitis (Dawson disease, SSPE)
Additional terms
References
- ↑ Lee VM et al, Neurodegenerative tauopathies Annu Rev Neurosci 24:1121-59, 2001 PMID: https://www.ncbi.nlm.nih.gov/pubmed/11520939
- ↑ Mailliot et al, Pathological tau phenotypes. The weight of mutations, polymorphisms, and differential neuronal vulnerabilities. PMID: https://www.ncbi.nlm.nih.gov/pubmed/11193138 Ann NY Acad Sci. 920:107-114, 2000
- ↑ Cairns NJ, Lee VM, Trojanowski JQ. The cytoskeleton in neurodegenerative diseases. J Pathol. 2004 Nov;204(4):438-49. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/15495240