Regional mitochondrial respiratory activity in Huntington's disease brain

J Neurochem. 1985 Jun;44(6):1948-50. doi: 10.1111/j.1471-4159.1985.tb07192.x.


This study investigated mitochondrial respiratory activity in Huntington's disease (HD) brain. Mitochondrial membranes from caudate and cortex of HD and non-HD autopsied brains were assayed for succinate oxidation, cytochrome oxidase activity, and cytochromes b, cc1, and aa3. There was a significant decrease in HD caudate mitochondrial respiration, cytochrome oxidase activity, and cytochrome aa3, whereas cytochromes b and cc1 were normal. These findings are consistent with the hypothesis that mitochondrial dysfunction may contribute to the localized hypometabolism and progressive atrophy of the HD caudate.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Brain / metabolism*
  • Electron Transport Complex IV / analysis
  • Humans
  • Huntington Disease / genetics
  • Huntington Disease / metabolism*
  • In Vitro Techniques
  • Mitochondria / metabolism*
  • Oxygen Consumption*


  • Electron Transport Complex IV