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

Item Type:Article
Title:Low stability of huntington muscle mitochondria against Ca2+ in R6/2 mice
Creators Name:Gizatullina, Z.Z. and Lindenberg, K.S. and Harjes, P. and Chen, Y. and Kosinski, C.M. and Landwehrmeyer, B.G. and Ludolph, A.C. and Striggow, F. and Zierz, S. and Gellerich, F.N.
Abstract:Objective: The aim of the present work was the detection of Mitochondrial dysfunction of Huntingdon'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.
Keywords:Animal Disease Models, Calcium, Drug Dose-Response Relationship, Huntington Disease, Inbred C57BL Mice, Muscle Mitochondria, Nerve Tissue Proteins, Nuclear Proteins, Oxygen Consumption, Respiration, Time Factors, Transgenic Mice, Trinucleotide Repeats, Animals, Mice
Source:Annals of Neurology
ISSN:0364-5134
Publisher:Wiley (U.S.A.)
Volume:59
Number:2
Page Range:407-411
Date:February 2006
Official Publication:https://doi.org/10.1002/ana.20754
PubMed:View item in PubMed

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