Date of Award
Spring 5-2012
Degree Type
Dissertation
Degree Name
Ph.D.
Degree Program
Applied Biopsychology
Department
Psychology
Major Professor
Gerald LaHoste
Second Advisor
Kevin Greve
Third Advisor
Michelle Martel
Fourth Advisor
Rodney Denis Soignier
Fifth Advisor
Laura Harrison
Abstract
Huntington's disease (HD) is a neuropsychiatric disorder characterized by choreiform movement of the limbs, cognitive disability, psychosis and dementia. It is untreatable, incurable, and ultimately fatal. HD is invariably associated with an abnormally long CAG expansion within the IT15 gene on human chromosome 4. Although the mutant huntingtin protein (mHtt) is ubiquitously expressed in HD patients, cellular degeneration occurs only in neurons within the striatum and cerebral cortex. The Ras homolog Rhes is expressed very selectively in the precise brain areas affected by HD. Recent work using cultured cells suggests that Rhes may be a co-factor with mHtt in cell death. However, there is controversy as to whether cell death underlies the symptoms of HD. We used a validated transgenic mouse model of HD crossed with Rhes knockout mice to show that the behavioral symptoms of HD are regulated by Rhes. HD/Rhes-/- mice showed greatly delayed expression of HD-like symptoms in this in vivo model. Drugs that block or inhibit the actions of Rhes may be useful as the first treatments for HD.
Recommended Citation
Baiamonte, Brandon A., "The effects of Rhes, a striatal specific protein, on the expression of behavioral and neuropathological symptoms in a transgenic mouse model of Huntington's disease" (2012). University of New Orleans Theses and Dissertations. 1424.
https://scholarworks.uno.edu/td/1424
Rights
The University of New Orleans and its agents retain the non-exclusive license to archive and make accessible this dissertation or thesis in whole or in part in all forms of media, now or hereafter known. The author retains all other ownership rights to the copyright of the thesis or dissertation.