Tempol, a superoxide dismutase mimetic agent, improves dystrophic phenotype in the diaphragm muscle of mdx mice

Abstract

Duchenne muscular dystrophy (DMD) is a progressive and lethal X‐linked myopathy, in which the oxidative stress and abnormal reactive oxygen species (ROS) production plays a key role in its pathophysiology. Despite intense efforts, no cure is currently available for DMD and glucocorticoids are the only effective drug to slow down disease progression. Thus, in the present study we investigated Tempol (a superoxide dismutase mimetic agent) effects on the diaphragm muscle of mdx mice, an experimental model of DMD. Mdx mice were randomly divided into two groups: mdxS, the control group receiving intraperitoneal (i.p.) injections of saline solution (100μL) and mdxT, the treated group receiving i.p. injections of Tempol (100 mg/kg). C57BL/10 mice were also used as controls. The results showed that Tempol treatment prevented muscle damage indicated by gain in muscle strength, decrease in the IgG uptake by muscle fibers and reduction of regenerated muscle fibers indicated by the central nuclei. It also had an effect on the inflammatory process, reducing the inflammatory area, macrophage staining and decreasing TNF‐α, IL‐1β and IL‐6 levels. Regarding oxidative stress, Tempol treatment was effective in reducing ROS and lipid peroxidation in the dystrophic diaphragm muscle. The findings presented here show that Tempol treatment improves dystrophic phenotype, supporting its use as a potential therapeutic strategy in DMD.Support or Funding InformationFAPESP #2017/01638‐0CAPES FAEPEXThis abstract is from the Experimental Biology 2019 Meeting. There is no full text article associated with this abstract published in The FASEB Journal.