Duchenne Muscular Dystrophy (DMD), the most common form of muscular dystrophy, is characterized by muscular wasting caused by dystrophin deficiency that ultimately ends in force reduction and premature death. In addition to primary genetic defect, several mechanisms contribute to DMD pathogenesis. Recently, antioxidant supplementation was shown to be effective in the treatment of multiple diseases including muscular dystrophy. Different mechanisms were hypothesized such as reduced hydroxyl radicals, nuclear factor-κB deactivation and NO protection from inactivation. Following these promising evidences, we investigated the effect of the administration of a mix of dietary natural polyphenols (ProAbe) on dystrophic mdx mice in term of muscular architecture and functionality. We observed a reduction of muscle fibrosis deposition and myofiber necrosis together with an amelioration of vascolarization. More importantly, the recovery of the morphological features of dystrophic muscle leads to an improvement of the endurance of treated dystrophic mice. Our data confirmed that ProAbe-based diet may represent a strategy to co-adjuvate the treatment of DMD.

Improvement of endurance of DMD animal model using natural polyphenols / C. Sitzia, A. Farini, F. Colleoni, F. Fortunato, P. Razini, S. Erratico, A. Tavelli, F. Fabrizi, M. Belicchi, M. Meregalli, G. Comi, Y. Torrente. - In: BIOMED RESEARCH INTERNATIONAL. - ISSN 2314-6141. - (2014), pp. 680615.-680615.1.

Improvement of endurance of DMD animal model using natural polyphenols

M. Belicchi;M. Meregalli;G. Comi;Y. Torrente
2014

Abstract

Duchenne Muscular Dystrophy (DMD), the most common form of muscular dystrophy, is characterized by muscular wasting caused by dystrophin deficiency that ultimately ends in force reduction and premature death. In addition to primary genetic defect, several mechanisms contribute to DMD pathogenesis. Recently, antioxidant supplementation was shown to be effective in the treatment of multiple diseases including muscular dystrophy. Different mechanisms were hypothesized such as reduced hydroxyl radicals, nuclear factor-κB deactivation and NO protection from inactivation. Following these promising evidences, we investigated the effect of the administration of a mix of dietary natural polyphenols (ProAbe) on dystrophic mdx mice in term of muscular architecture and functionality. We observed a reduction of muscle fibrosis deposition and myofiber necrosis together with an amelioration of vascolarization. More importantly, the recovery of the morphological features of dystrophic muscle leads to an improvement of the endurance of treated dystrophic mice. Our data confirmed that ProAbe-based diet may represent a strategy to co-adjuvate the treatment of DMD.
Settore MED/26 - Neurologia
2014
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/250079
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