SEARCH

SEARCH BY CITATION

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