Objective
Oxidative stress contributes to
myonecrosis in the dystrophin-deficient fibers of
m>mdxm>
mice and in Duchenne鈥檚
muscular dystrophy. We exa
mined the effects of ascorbic acid (AA), an antioxidant and free radical scavenger, on the dystrophic diaphrag
m muscle.
Methods
m>Mdxm> mice (14 d old) received AA for 14 d. Control m>mdxm> mice received saline. The muscle damage was visualized by the penetration of Evans blue dye into myofibers and the extent of inflammation was assessed by histologic analysis. Creatine kinase levels were measured for the biochemical evaluation of muscle fiber degeneration. The levels of tumor necrosis factor-伪 (a聽proinflammatory cytokine) and 4-hydroxynonenal (a marker of lipid peroxidation) were analyzed by immunoblotting.
Results
Ascorbic acid decreased creatine kinase levels, myonecrosis, inflammation, and the levels of tumor necrosis factor-伪 and 4-hydroxynonenal.
Conclusion
The present results suggest that AA plays a protective role in dystrophic muscle degeneration, possibly by decreasing reactive oxygen species, and support further investigations of AA as a potential therapy for dystrophinopathies.