期刊论文详细信息
Antioxidants 卷:6
Tempol Supplementation Restores Diaphragm Force and Metabolic Enzyme Activities in mdx Mice
David P. Burns1  Ken D. O’Halloran1  James Healy1  Clement Rieux1  Izza Ali1  Greg Jasionek1 
[1] Department of Physiology, University College Cork, Western Gateway Building, Western Road, Cork T12 XF62, Ireland;
关键词: Duchenne muscular dystrophy;    mdx;    tempol;    oxidative stress;    diaphragm;    antioxidant;   
DOI  :  10.3390/antiox6040101
来源: DOAJ
【 摘 要 】

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.

【 授权许可】

Unknown   

  文献评价指标  
  下载次数:0次 浏览次数:0次