Low stability of huntington muscle Mitochondria against Ca2+ in R6/2 mice
Article first published online: 25 JAN 2006
Copyright © 2006 American Neurological Association
Annals of Neurology
Volume 59, Issue 2, pages 407–411, February 2006
How to Cite
Gizatullina, Z. Z., Lindenberg, K. S., Harjes, P., Chen, Y., Kosinski, C. M., Landwehrmeyer, B. G., Ludolph, A. C., Striggow, F., Zierz, S. and Gellerich, F. N. (2006), Low stability of huntington muscle Mitochondria against Ca2+ in R6/2 mice. Ann Neurol., 59: 407–411. doi: 10.1002/ana.20754
- Issue published online: 25 JAN 2006
- Article first published online: 25 JAN 2006
- Manuscript Accepted: 22 OCT 2005
- Manuscript Revised: 2 SEP 2005
- Manuscript Received: 13 JUN 2005
- DFG. Grant Number: Ge 664/11
The aim of the present work was the detection of Mitochondrial dysfunction of Huntington's disease (HD).
We investigated muscle and muscle mitochondria of 14- to 16-week-old R6/2 mice in comparison with wild-type mice.
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.
Data suggest that the decreased stability of HD mitochondria against Ca2+ contributes to energetic depression and cell atrophy. Ann Neurol 2006;59:407–411