Abstract
Tau protein is the major component of the intracellular filamentous deposits that define a number of neurodegenerative diseases. They include the largely sporadic Alzheimer's disease, progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), Pick's disease (PiD), argyrophilic grain disease, as well as the inherited frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17). The identification of mutations in Tau as the cause of FTDP-17 established that dysfunction or misregulation of tau protein is sufficient to cause neurodegeneration and dementia. At an experimental level, the new understanding is leading to the development of good transgenic animal models of the tauopathies.
MeSH terms
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Alternative Splicing
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Amyloid / chemistry
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Amyloid / metabolism
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Amyloid / ultrastructure
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Animals
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Brain / metabolism
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Brain / pathology
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Dementia / etiology
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Dementia / genetics
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Dementia / physiopathology
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Disease Models, Animal
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Glycosaminoglycans / chemistry
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Glycosaminoglycans / metabolism
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Humans
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Mice
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Mice, Transgenic
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Microscopy, Electron
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Microtubules / metabolism
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Mutation / genetics
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Nerve Tissue Proteins / chemistry
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Nerve Tissue Proteins / genetics*
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Neurodegenerative Diseases / etiology
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Neurodegenerative Diseases / genetics
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Parkinsonian Disorders / etiology
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Parkinsonian Disorders / genetics
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Parkinsonian Disorders / physiopathology
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Pick Disease of the Brain / etiology
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Pick Disease of the Brain / genetics
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Protein Binding
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Protein Isoforms / chemistry
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Protein Isoforms / genetics
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Supranuclear Palsy, Progressive / etiology
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Supranuclear Palsy, Progressive / genetics
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Tauopathies / etiology
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Tauopathies / genetics*
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tau Proteins
Substances
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Amyloid
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Glycosaminoglycans
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MAPT protein, human
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Nerve Tissue Proteins
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Protein Isoforms
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tau Proteins