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Antioxidants 2017, 6(4), 101;

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

Department of Physiology, University College Cork, Western Gateway Building, Western Road, Cork T12 XF62, Ireland
Author to whom correspondence should be addressed.
Received: 17 October 2017 / Revised: 19 November 2017 / Accepted: 28 November 2017 / Published: 6 December 2017
(This article belongs to the Special Issue Exercise Induced Muscle Damage and Oxidative Stress)
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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 mdx 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 mdx diaphragm. These findings may have relevance in the search for therapeutic strategies in neuromuscular disease. View Full-Text
Keywords: Duchenne muscular dystrophy; mdx; tempol; oxidative stress; diaphragm; antioxidant Duchenne muscular dystrophy; mdx; tempol; oxidative stress; diaphragm; antioxidant

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Burns, D.P.; Ali, I.; Rieux, C.; Healy, J.; Jasionek, G.; O’Halloran, K.D. Tempol Supplementation Restores Diaphragm Force and Metabolic Enzyme Activities in mdx Mice. Antioxidants 2017, 6, 101.

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