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Ann Neurol. 2006 Feb;59(2):407-11.

Low stability of Huntington muscle mitochondria against Ca2+ in R6/2 mice.

Author information

1
KeyNeurotek AG, ZENIT Technology Park, Magdeburg, Germany.

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.

PMID:
16437579
DOI:
10.1002/ana.20754
[Indexed for MEDLINE]

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