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Publication : Dystrophic phenotype improvement in the diaphragm muscle of mdx mice by diacerhein.

First Author  Mâncio RD Year  2017
Journal  PLoS One Volume  12
Issue  8 Pages  e0182449
PubMed ID  28787441 Mgi Jnum  J:248583
Mgi Id  MGI:5918071 Doi  10.1371/journal.pone.0182449
Citation  Mancio RD, et al. (2017) Dystrophic phenotype improvement in the diaphragm muscle of mdx mice by diacerhein. PLoS One 12(8):e0182449
abstractText  Chronic inflammation and oxidative stress are striking features of Duchenne muscular dystrophy disease. Diacerhein is an anthraquinone, which exhibits anti-inflammatory and antioxidant properties. Based on their actions, the present study evaluated the effects of diacerhein against myonecrosis, oxidative stress and inflammatory response in the diaphragm muscle of mdx mice and compared these results to current treatment widely used in DMD patients, with a main focus on the impact of prednisone. The results demonstrated that diacerhein treatment prevented muscle damage indicated by a decrease in the IgG uptake by muscle fibers, lower CK levels in serum, reduction of fibers with central nuclei with a concomitant increase in fibers with peripheral nuclei. It also had an effect on the inflammatory process, decreasing the inflammatory area, macrophage staining and TNF-alpha and IL-1beta content. Regarding oxidative stress, diacerhein treatment was effective in reducing the ROS and lipid peroxidation in the diaphragm muscle from mdx mice. Compared to prednisone treatment, our findings demonstrated that diacerhein treatment improved the dystrophic phenotype in the diaphragm muscle of mdx mice similar to that of glucocorticoid therapy. In this respect, this work suggests that diacerhein has a potential use as an alternative drug in dystrophinopathy treatment and recommends that its anti-inflammatory and antioxidants properties in the dystrophic muscle should be better understood.
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