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• W2053985220 abstract "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 Ca 2+ accumulation experiments, a decreased stability of HD mitochondria against Ca 2+ ‐induced permeability transition was detected. Complex I–dependent respiration of HD mitochondria was more sensitive to inhibition by adding 10μm Ca 2+ than wild‐type mitochondria. Interpretation Data suggest that the decreased stability of HD mitochondria against Ca 2+ contributes to energetic depression and cell atrophy. Ann Neurol 2006;59:407–411" @default.
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• W2053985220 date "2006-01-25" @default.
• W2053985220 modified "2023-10-18" @default.
• W2053985220 title "Low stability of huntington muscle Mitochondria against Ca²⁺ in R6/2 mice" @default.
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• W2053985220 doi "https://doi.org/10.1002/ana.20754" @default.
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