Tempol Supplementation Restores Diaphragm Force and Metabolic Enzyme Activities in mdx Mice

Duchenne muscular dystrophy (DMD) is characterized by striated muscle weakness, cardiomyopathy, and respiratory failure. Since oxidative stress is recognized as a secondary pathology in DMD, the efficacy of antioxidant intervention, using the superoxide scavenger tempol, was examined on functional a...

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Bibliographic Details
Published in:Antioxidants 2017-12, Vol.6 (4), p.101
Main Authors: Burns, David P, Ali, Izza, Rieux, Clement, Healy, James, Jasionek, Greg, O'Halloran, Ken D
Format: Article
Language:English
Subjects:
antioxidant
Antioxidants
Cardiomyopathy
Citrate synthase
Dehydrogenases
Diaphragm
Drinking water
Duchenne muscular dystrophy
Duchenne's muscular dystrophy
Dystrophin
Enzymatic activity
Enzymes
L-Lactate dehydrogenase
Lactic acid
mdx
Metabolism
Muscles
Oxidative stress
Phosphofructokinase
Respiration
Rodents
Skeletal muscle
Spectrophotometry
Superoxide
Supplements
Tempol
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Summary:Duchenne muscular dystrophy (DMD) is characterized by striated muscle weakness, cardiomyopathy, and respiratory failure. Since oxidative stress is recognized as a secondary pathology in DMD, the efficacy of antioxidant intervention, using the superoxide scavenger tempol, was examined on functional and biochemical status of dystrophin-deficient diaphragm muscle. Diaphragm muscle function was assessed, ex vivo, in adult male wild-type and dystrophin-deficient mice, with and without a 14-day antioxidant intervention. The enzymatic activities of muscle citrate synthase, phosphofructokinase, and lactate dehydrogenase were assessed using spectrophotometric assays. Dystrophic diaphragm displayed mechanical dysfunction and altered biochemical status. Chronic tempol supplementation in the drinking water increased diaphragm functional capacity and citrate synthase and lactate dehydrogenase enzymatic activities, restoring all values to wild-type levels. Chronic supplementation with tempol recovers force-generating capacity and metabolic enzyme activity in diaphragm. These findings may have relevance in the search for therapeutic strategies in neuromuscular disease.
ISSN:2076-3921
2076-3921
DOI:10.3390/antiox6040101