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

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

Research output: Contribution to journalArticlepeer-review

75 Scopus citations

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.

Original languageEnglish
Pages (from-to)1870-1876
Number of pages7
JournalBrain
Volume128
Issue number8
DOIs
StatePublished - Aug 2005
Externally publishedYes

Keywords

  • Amyotrophic lateral sclerosis
  • Mitochondria
  • Neurogenic atrophies

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