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tauopathy
Disorders characterized by abnormal deposition of the mt-tau:
- Alzheimer's disease
- frontotemporal dementias
- frontotemporal dementia with parkinsonism (FTDP-17)
- Pick's disease
- 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
Related
microtubule-associated protein tau (neurofibrillary tangle protein, paired helical filament-tau, PHF-tau, MAPT, MTBT1, m-tau, mtau)
Specific
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)
General
neurodegenerative disease
References
- Lee VM et al,
Neurodegenerative tauopathies
Annu Rev Neurosci 24:1121-59, 2001
PMID: 11520939
- Mailliot et al,
Pathological tau phenotypes. The weight of mutations,
polymorphisms, and differential neuronal vulnerabilities.
PMID: 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: 15495240