Peer-reviewed veterinary case report
Low stability of Huntington muscle mitochondria against Ca2+ in R6/2 mice.
- Journal:
- Annals of neurology
- Year:
- 2006
- Authors:
- Gizatullina, Zemfira Z et al.
- Affiliation:
- KeyNeurotek AG · Germany
- Species:
- rodent
Abstract
OBJECTIVE: The aim of the present work was the detection of Mitochondrial dysfunction of Huntington's disease (HD). METHODS: We investigated muscle and muscle mitochondria of 14- to 16-week-old R6/2 mice in comparison with wild-type mice. RESULTS: 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. INTERPRETATION: Data suggest that the decreased stability of HD mitochondria against Ca2+ contributes to energetic depression and cell atrophy.
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Search related cases →Original publication: https://pubmed.ncbi.nlm.nih.gov/16437579/