Low stability of Huntington muscle mitochondria against Ca2+ in R6/2 mice.
case_control · Level III
Where this comes from
- Record sourced from PubMed, PMID 16437579.
- No licence information is recorded for this record.
- Because redistribution is not established, this page shows the abstract only. Follow the links below for the full text.
Abstract
The aim of the present work was the detection of Mitochondrial dysfunction of Huntington's disease (HD). We investigated muscle and muscle mitochondria of 14- to 16-week-old R6/2 mice in comparison with wild-type mice. Atrophic fibers, increased fuchsinophilic aggregates, and reduced cytochrome c oxidase (15%) were found in HD muscle. With swelling measurements and Ca2+ accumulation experiments, a decreased stability of HD mitochondria against Ca2+-induced permeability transition was detected. Complex I-dependent respiration of HD mitochondria was more sensitive to inhibition by adding 10 microm Ca2+ than wild-type mitochondria. Data suggest that the decreased stability of HD mitochondria against Ca2+ contributes to energetic depression and cell atrophy.
Medical subject headings
- Calcium
- Huntington Disease
- Mitochondria, Muscle