Key result
Suramin protects against diaphragm fatigue in mdx mice by altering metalloproteinase-9 activity and increasing beta-dystroglycan.
Why the study?
Muscle fiber degeneration and fibrosis in Duchenne muscular dystrophy and mdx mice lead to cardiorespiratory failure, and the effects of suramin on metalloproteinases and diaphragm function were unclear.
Hypothesis-generating for suramin in dystrophinopathies; leaves open translation from mdx model to patients.
INTRODUCTION: In Duchenne muscular dystrophy and in the mdx mouse, muscle fiber degeneration and subsequent fibrosis lead to cardiorespiratory failure. Previously, we demonstrated that the anti-fibrotic agent suramin was effective in decreasing fibrosis in mdx muscles. In this study, we were interested to see whether suramin could affect metalloproteinases (MMP) and improve the functional activity of the mdx diaphragm muscle. METHODS: Zymography was performed to evaluate MMP-2 and MMP-9 activity. Western blotting was used to analyze the levels of beta-dystroglycan. Muscle function was assessed in hemidiaphragm in vitro preparations. RESULTS: We found that suramin affects metalloproteinase-9 activity and increases beta-dystroglycan. Furthermore, suramin also protects against diaphragm muscle fatigue over time. CONCLUSIONS: These results show the potential benefits of suramin in maintaining the structure of the dystrophin-glycoprotein complex.
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Taniguti et al. (2012) studied Duchenne muscular dystrophy (mdx mouse model). Suramin was evaluated on metalloproteinase-9 activity, beta-dystroglycan levels, and diaphragm muscle fatigue. Suramin affected metalloproteinase-9 activity, increased beta-dystroglycan levels, and protected against diaphragm muscle fatigue over time in the dystrophin-deficient mdx mouse.
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