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Brain Advance Access first published online on May 18, 2005
This version published online on May 25, 2005
Brain, doi:10.1093/brain/awh540
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© The Author (2005). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For Permissions, please email: journals.permissions@oupjournals.org
Received February 17, 2005
Revised April 5, 2005
Accepted April 12, 2005

Article

Mitochondrial changes in skeletal muscle in amyotrophic lateral sclerosis and other neurogenic atrophies

Anna Krasnianski 1, Marcus Deschauer 1, Stephan Neudecker 1, Frank N. Gellerich 1, Tobias Müller 1, Benedikt G. Schoser 2, Michael Krasnianski 1, and Stephan Zierz 1*

1 Department of Neurology, Martin-Luther-University Halle-Wittenberg, Halle (Saale), Germany
2 Department of Neurology, University of Hamburg, Hamburg, Germany

* To whom correspondence should be addressed.
Stephan Zierz, E-mail: sekretariat.neurologie{at}medizin.uni-halle.de


  Abstract

Previous findings suggested specific mitochondrial dysfunction in skeletal muscle of patients with amyotrophic lateral sclerosis (ALS). To answer the question of whether the dysfunction is specific, we investigated the histochemical distribution of mitochondrial marker activities, the ratio of mitochondrial (mt) versus nuclear (n) DNA, and the activities of citrate synthase (CS) and respiratory chain enzymes in muscle biopsies of 24 patients with sporadic ALS. The data were compared with those in 23 patients with other neurogenic atrophies (NAs), and 21 healthy controls. Muscle histology revealed similar signs of focally diminished mitochondrial oxidation activity in muscle fibres in both diseased groups. There was only minimal decline of mt/nDNA ratios in ALS and NA patients in comparison with healthy controls. The specific activities of mitochondrial markers CS and succinate dehydrogenase were significantly increased in both ALS and NA patients. The specific activities of respiratory chain enzymes were not significantly different in all three groups. It is concluded that the histochemical, biochemical and molecular mitochondrial changes in muscle are not specific for ALS, but accompany other NAs as well.

Keywords: amyotrophic lateral sclerosis; neurogenic atrophies; mitochondria.
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