Normal platelet mitochondrial complex I activity in Huntington's disease

Neurobiol Dis. 2007 Jul;27(1):99-101. doi: 10.1016/j.nbd.2007.04.008. Epub 2007 May 5.


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.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Blood Platelets / enzymology*
  • Electron Transport Complex I / metabolism*
  • Electron Transport Complex III / metabolism
  • Enzyme Activation
  • Female
  • Humans
  • Huntington Disease / metabolism*
  • Male
  • Middle Aged
  • Mitochondria / enzymology*


  • Electron Transport Complex I
  • Electron Transport Complex III