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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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Autores principales: Burns, David P., Ali, Izza, Rieux, Clement, Healy, James, Jasionek, Greg, O’Halloran, Ken D.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5745511/
https://www.ncbi.nlm.nih.gov/pubmed/29210997
http://dx.doi.org/10.3390/antiox6040101
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author Burns, David P.
Ali, Izza
Rieux, Clement
Healy, James
Jasionek, Greg
O’Halloran, Ken D.
author_facet Burns, David P.
Ali, Izza
Rieux, Clement
Healy, James
Jasionek, Greg
O’Halloran, Ken D.
author_sort Burns, David P.
collection PubMed
description 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.
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spelling pubmed-57455112018-01-02 Tempol Supplementation Restores Diaphragm Force and Metabolic Enzyme Activities in mdx Mice Burns, David P. Ali, Izza Rieux, Clement Healy, James Jasionek, Greg O’Halloran, Ken D. Antioxidants (Basel) Article 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. MDPI 2017-12-06 /pmc/articles/PMC5745511/ /pubmed/29210997 http://dx.doi.org/10.3390/antiox6040101 Text en © 2017 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Burns, David P.
Ali, Izza
Rieux, Clement
Healy, James
Jasionek, Greg
O’Halloran, Ken D.
Tempol Supplementation Restores Diaphragm Force and Metabolic Enzyme Activities in mdx Mice
title Tempol Supplementation Restores Diaphragm Force and Metabolic Enzyme Activities in mdx Mice
title_full Tempol Supplementation Restores Diaphragm Force and Metabolic Enzyme Activities in mdx Mice
title_fullStr Tempol Supplementation Restores Diaphragm Force and Metabolic Enzyme Activities in mdx Mice
title_full_unstemmed Tempol Supplementation Restores Diaphragm Force and Metabolic Enzyme Activities in mdx Mice
title_short Tempol Supplementation Restores Diaphragm Force and Metabolic Enzyme Activities in mdx Mice
title_sort tempol supplementation restores diaphragm force and metabolic enzyme activities in mdx mice
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5745511/
https://www.ncbi.nlm.nih.gov/pubmed/29210997
http://dx.doi.org/10.3390/antiox6040101
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