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Normal platelet mitochondrial complex I activity in Huntington's disease
Authors:Powers William J  Haas Richard H  Le Thuy  Videen Tom O  Hershey Tamara  McGee-Minnich Lori  Perlmutter Joel S
Affiliation:Department of Neurology, Washington University School of Medicine, St Louis, MO 63110, USA.
Abstract:Two small case series of platelet mitochondrial complex I activity assays in Huntington's Disease (HD) report discrepant results. We measured platelet complex I and complex I/III activity in 21 subjects with early gene-positive HD and 14 age-matched controls. The 21 participants with HD that we studied are greater than the total of 16 in the two previously published of platelet ETS activity in HD. Reductions > 10% were excluded with 80% confidence. A systemic defect in complex I activity is not present in early HD when striatal neuronal degeneration is already present.
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