Low stability of huntington muscle Mitochondria against Ca2+ in R6/2 mice

Authors


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 10μm Ca2+ than wild-type mitochondria.

Interpretation

Data suggest that the decreased stability of HD mitochondria against Ca2+ contributes to energetic depression and cell atrophy. Ann Neurol 2006;59:407–411

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