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Huntington’s Disease (HD) is a neurodegenerative disorder for which no treatment has been identified to prevent, limit, or cure symptoms of involuntary choreiform movements, behavioral abnormalities, dementia, and motor dysfunction as a result of neuronal loss in the striatum and cerebral cortex. Although it is known that HD is caused by mutations in the huntingtin gene, the causality of this mutation in the mechanism of neuronal loss remains unknown. Compelling evidence suggests that increased oxidative stress and mitochondrial dysfunction contribute to the pathophysiology of HD, thus, a better understanding of why mitochondrial dysfunction may arise during HD neurodegeneration could provide the basis for the development of new pharmacological interventions to delay disease onset and progression. We apply cellular, biochemical and molecular approaches using both in vitro and in vivo experimental models of HD. We also test small molecules with antioxidant activity as interventions to prevent onset and/or progression of the disease. Because aging is the main risk factor for chronic diseases and is associated with increased DNA damage and decreased DNA repair capacity, we are also studying the role of mitochondria in diabetes, an age-associated disorder, and testing pharmacological interventions that may protect mtDNA integrity and mitochondrial function.
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