Journal
PARKINSONISM & RELATED DISORDERS
Volume 22, Issue -, Pages S29-S33Publisher
ELSEVIER SCI LTD
DOI: 10.1016/j.parkreldis.2015.09.020
Keywords
Progressive supranuclear palsy; Corticobasal degeneration; Corticobasal syndrome tauopathy; Pick's disease; Frontotemporal dementia; Frontotemporal lobar degeneration; Primary progressive aphasia; MAPT mutation; Argyrophilic grain disease; Primary age related tauopathy
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Funding
- NIA NIH HHS [P01 AG017586] Funding Source: Medline
- NINDS NIH HHS [K23 NS088341, NS088341] Funding Source: Medline
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Tauopathies are a class of neurodegenerative disorders characterized by neuronal and/or glial inclusions composed of the microtubule-binding protein, tau. Several lines of evidence suggest tau aggregation is central to the neurodegenerative process in tauopathies. First, recent animal and cell model studies find abnormally-modified tau alone may be transmitted between adjacent neurons and spread to anatomically connected brain regions to recapitulate human disease. Further, staging efforts in human autopsy cases suggest a sequential distribution of tau aggregation in the central nervous system that could reflect this observed cell-to-cell transmission of pathogenic tau species in animal models. Finally, pathogenic mutations in the MAPT gene encoding tau protein cause hereditary forms of tauopathy. Clinically, tauopathies can present with a range of phenotypes that include both movement- and cognitive/behavioral-disorders (i.e. frontotemporal dementia spectrum disorders) or non-specific amnestic symptoms in advanced age. A major limitation is that current clinical diagnostic criteria for these disorders do not reliably differentiate underlying tauopathy from other neurodegenerative diseases, such as TDP-43 proteinopathies. Thus, current research efforts are focused on improving the ante mortem diagnosis of tauopathies, including pre-clinical stages of disease, as many therapeutic strategies for emerging disease-modifying therapies focus on preventing abnormal folding and spread of tau pathology. (C) 2015 Elsevier Ltd. All rights reserved.
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