Journal
EUROPEAN JOURNAL OF NEUROLOGY
Volume 16, Issue 3, Pages 297-309Publisher
WILEY-BLACKWELL PUBLISHING, INC
DOI: 10.1111/j.1468-1331.2008.02513.x
Keywords
corticobasal degeneration; frontotemporal dementia with parkinsonism linked to chromosome 17; microtubule-associated protein tau; multiple system atrophy; Parkinson disease; parkinsonism; progressive supranuclear palsy; tauopathies
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Funding
- Morris K. Udall Center of Excellence in Parkinson's Disease Research [P50-NS40256]
- Pacific Alzheimer Research Foundation [C06-01]
- NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE [P50NS040256] Funding Source: NIH RePORTER
- NATIONAL INSTITUTE ON AGING [P50AG008702] Funding Source: NIH RePORTER
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Tauopathies with parkinsonism represent a spectrum of disease entities unified by the pathologic accumulation of hyperphosphorylated tau protein fragments within the central nervous system. These pathologic characteristics suggest shared pathogenetic pathways and possible molecular targets for disease-modifying therapeutic interventions. Natural history studies, for instance, in progressive supranuclear palsy, frontotemporal dementia with parkinsonism linked to chromosome 17, corticobasal degeneration, and Niemann-Pick disease type C as well as in amyotrophic lateral sclerosis/Parkinson-dementia complex permit clinical characterization of the disease phenotypes and are crucial to the development and validation of biological markers for differential diagnostics and disease monitoring, for example, by use of neuroimaging or proteomic approaches. The wide pathologic and clinical spectrum of the tauopathies with parkinsonism is reviewed in this article, and perspectives on future advances in the understanding of the pathogenesis are given, together with potential therapeutic strategies.
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