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

摘要: Duchenne muscular dystrophy (DMD) is characterized by striated muscle weakness, cardiomyopathy, and respiratory failure. Since oxidative stress recognized as a secondary pathology in DMD, the efficacy of antioxidant intervention, using superoxide scavenger tempol, was examined on functional biochemical status dystrophin-deficient diaphragm muscle. Diaphragm function assessed, ex vivo, adult male wild-type mdx mice, with without 14-day intervention. The enzymatic activities citrate synthase, phosphofructokinase, lactate dehydrogenase were assessed spectrophotometric assays. Dystrophic displayed mechanical dysfunction altered status. Chronic tempol supplementation drinking water increased capacity synthase activities, restoring all values to levels. recovers force-generating metabolic enzyme activity diaphragm. These findings may have relevance search for therapeutic strategies neuromuscular disease.