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Low stability of huntington muscle Mitochondria against Ca 2+ in R6/2 mice
Author(s) -
Gizatullina Zemfira Z.,
Lindenberg Katrin S.,
Harjes Phoebe,
Chen Ying,
Kosinski Christoph M.,
Landwehrmeyer Bernhard G.,
Ludolph Albert C.,
Striggow Frank,
Zierz Stephan,
Gellerich Frank N.
Publication year - 2006
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.20754
Subject(s) - mitochondrion , huntington's disease , cytochrome c oxidase , atrophy , mitochondrial permeability transition pore , medicine , chemistry , cytochrome c , endocrinology , biology , apoptosis , microbiology and biotechnology , biochemistry , disease , programmed cell death
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