Tempol supplementation restores diaphragm force and metabolic enzyme activities in mdx mice

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dc.contributor.authorBurns, David P.
dc.contributor.authorAli, Izza
dc.contributor.authorRieux, Clement
dc.contributor.authorHealy, James
dc.contributor.authorJasionek, Greg
dc.contributor.authorO'Halloran, Ken D.
dc.contributor.funderUniversity College Cork
dc.contributor.funderEuropean Commission
dc.date.accessioned2018-02-06T13:36:29Z
dc.date.available2018-02-06T13:36:29Z
dc.date.issued2017
dc.description.abstractDuchenne 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.en
dc.description.sponsorshipEuropean Union (Erasmus)en
dc.description.statusPeer revieweden
dc.description.versionPublished Versionen
dc.format.mimetypeapplication/pdfen
dc.identifier.articleid101
dc.identifier.citationBurns, D., Ali, I., Rieux, C., Healy, J., Jasionek, G. and O’Halloran, K. (2017) 'Tempol supplementation restores diaphragm force and metabolic enzyme activities in mdx mice', Antioxidants, 6(4), 101 (16pp). doi: 10.3390/antiox6040101en
dc.identifier.doi10.3390/antiox6040101
dc.identifier.endpage16
dc.identifier.issued4
dc.identifier.journaltitleAntioxidantsen
dc.identifier.startpage1
dc.identifier.urihttps://hdl.handle.net/10468/5399
dc.identifier.volume6
dc.language.isoenen
dc.publisherMDPI AGen
dc.relation.urihttp://www.mdpi.com/2076-3921/6/4/101
dc.rights© 2017, 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/).en
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/
dc.subjectDuchenne muscular dystrophyen
dc.subjectmdxen
dc.subjectTempolen
dc.subjectOxidative stressen
dc.subjectDiaphragmen
dc.subjectAntioxidanten
dc.titleTempol supplementation restores diaphragm force and metabolic enzyme activities in mdx miceen
dc.typeArticle (peer-reviewed)en
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