title: Normal platelet mitochondrial complex I activity in Huntington's disease.
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.
authors.affiliation: Department of Neurology, Washington University School of Medicine, St Louis, MO 63110, USA.
authors.forename: William J
authors.initials: WJ
authors.lastname: Powers
authors.forename: Richard H
authors.initials: RH
authors.lastname: Haas
authors.forename: Thuy
authors.initials: T
authors.lastname: Le
authors.forename: Tom O
authors.initials: TO
authors.lastname: Videen
authors.forename: Tamara
authors.initials: T
authors.lastname: Hershey
authors.forename: Lori
authors.initials: L
authors.lastname: McGee-Minnich
authors.forename: Joel S
authors.initials: JS
authors.lastname: Perlmutter
mesh_terms: D000328:Adult; D001792:Blood Platelets / Q000201:enzymology*; D042967:Electron Transport Complex I / Q000378:metabolism*; D014450:Electron Transport Complex III / Q000378:metabolism; D004789:Enzyme Activation; D005260:Female; D006801:Humans; D006816:Huntington Disease / Q000378:metabolism*; D008297:Male; D008875:Middle Aged; D008928:Mitochondria / Q000201:enzymology*
chemical_list: D042967:Electron Transport Complex I; D014450:Electron Transport Complex III