This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Huntington's disease (HD) is a devastating disease of the brain. HD usually begins between the ages of 30 to 50, although rarely it can begin earlier or later, and progresses to death within 15-25 years. HD is inherited as an autosomal dominant, neurodegenerative disorder caused by a CAG repeat expansion in a gene called 'huntington'. HD affects approximately 30,000 Americans and is characterized by insidious development of motor, cognitive and psychiatric disturbances with an inexorable progression towards death. There is a 50% risk that each child of a parent will inherit the disease. Ethyl-EPA (ethyl-eicosapentaenoic acid) is a semi-synthetic, highly purified form of the fatty acid EPA attached to an ethyl group. The ethyl group is cleaved from the EPA in the gut and the EPA is absorbed into the blood stream. Although the exact mechanism of action of ethyl-EPA in HD is not known, it is thought to stabilize mitochondrial integrity and improve neuronal dysfunction. This could occur directly and/or indirectly via several pathways, which have been shown to be affected by EPA in pre-clinical studies. EPA has been implicated in various studies to trigger induction of expression of certain enzymes and regulatory factors though the exact relevance of these studies to its potential use as a therapeutic in HD is not yet known. This protocol will compare placebo to the effect of ethyl-EPA in subjects with Huntington's disease.
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