Progressive Supranuclear Palsy (PSP) belongs to a family of neurodegenerative disorders referred to as "tauopathies", and is the most frequent atypical neurodegenerative parkinsonian disorder, with an estimated prevalence of 4-6.5/ 100,000 in the general population. Symptoms of the disease usually appear between 50- 80 years of age, and include postural instability, supranuclear vertical gaze palsy, severe speech and swallowing difficulties, and alterations in mood and behavior. The disease is progressively debilitating and patients die within a few years after the onset of symptoms. To date there is no cure for PSP, and development of therapies for the disease require further understanding of the mechanisms underlying the neuropathological changes associated with PSP. A mouse model recapitulating the phenotypic features of the disease would therefore be an invaluable tool to understand the disease process and test new therapeutic strategies. Such mouse model has not been established yet. The main goal of this application is to generate a mouse model for PSP. The availability of a mouse model for PSP will allow not only further understanding of neuropathological changes that occur in tauopathies, but also the possibility of developing new therapeutic interventions for this devastating disorder.

Public Health Relevance

Progressive Supranuclear Palsy (PSP) belongs to a family of neurodegenerative disorders called "tauopathies" and is the most frequent atypical neurodegenerative Parkinsonian disorder. The disease is inevitably fatal with death occurring a few years after symptoms appear. Generation and analyses of a mouse model for this disorder will provide insights for potential new therapeutic interventions for PSP and for other tauopathies.

Agency
National Institute of Health (NIH)
Institute
National Institute of Neurological Disorders and Stroke (NINDS)
Type
Small Research Grants (R03)
Project #
5R03NS071207-02
Application #
8259429
Study Section
Cell Death in Neurodegeneration Study Section (CDIN)
Program Officer
Sieber, Beth-Anne
Project Start
2011-07-01
Project End
2014-04-30
Budget Start
2012-05-01
Budget End
2014-04-30
Support Year
2
Fiscal Year
2012
Total Cost
$74,959
Indirect Cost
$24,959
Name
University of Tennessee Health Science Center
Department
Physiology
Type
Schools of Medicine
DUNS #
941884009
City
Memphis
State
TN
Country
United States
Zip Code
38163