Brain Advance Access originally published online on May 18, 2005
Brain 2005 128(8):1870-1876; doi:10.1093/brain/awh540
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Mitochondrial changes in skeletal muscle in amyotrophic lateral sclerosis and other neurogenic atrophies
1 Department of Neurology, Martin-Luther-University Halle-Wittenberg, Halle (Saale) and 2 Department of Neurology, University of Hamburg, Hamburg, Germany
Correspondence to: Stephan Zierz, MD, Klinik und Poliklinik für Neurologie, Martin-Luther-Universität Halle-Wittenberg, Ernst-Grube Str. 40, D-06097 Halle (Saale), Germany E-mail: sekretariat.neurologie{at}medizin.uni-halle.de
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.
Key Words: amyotrophic lateral sclerosis; neurogenic atrophies; mitochondria
Abbreviations: ALS = amyotrophic lateral sclerosis; COX = cytochrome c oxidase; CPEO = chronic progressive external ophthalmoplegia; CS = citrate synthase; HC = healthy control; mt = mitochondrial; n = nuclear; NA = neurogenic atrophy; NCP = non-collagen protein; PGI = phosphoglucose isomerase; SALS = sporadic amyotrophic lateral sclerosis; SDH = succinate dehydrogenase
Received February 17, 2005. Revised April 5, 2005. Accepted April 12, 2005.
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 A. Krasnianski, M. Deschauer, M. Krasnianski, and S. Zierz Reply to 'Mitochondrial changes in skeletal muscle in amyotrophic lateral sclerosis and other neurogenic atrophies--a comment'. Brain, March 1, 2006; 129(Pt 3): E41 - E41. [Full Text] [PDF]
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