Regional mitochondrial respiratory activity in Huntington's disease brain.
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.
Brennan
W A
WA
Bird
E D
ED
Aprille
J R
JR
D001921:Brain / Q000378:metabolism*; D003576:Electron Transport Complex IV / Q000032:analysis; D006801:Humans; D006816:Huntington Disease / Q000235:genetics / Q000378:metabolism*; D066298:In Vitro Techniques; D008928:Mitochondria / Q000378:metabolism*; D010101:Oxygen Consumption
D003576:Electron Transport Complex IV