Improvement of Endurance of DMD Animal Model Using Natural Polyphenols

• Published in: BioMed research international 2015-01, Vol.2015 (2015), p.1-17
• Main Authors: Meregalli, Mirella, Comi, Giacomo P., Torrente, Yvan, Fabrizi, Francesco, Tavelli, Alessandro, Erratico, Silvia, Razini, Paola, Fortunato, Francesco, Colleoni, Federica, Farini, Andrea, Sitzia, Clementina, Belicchi, Marzia
• Format: Article
• Language: English
• Subjects: Animals; Antioxidants; Cells, Cultured; Chronic illnesses; Disease Models, Animal; Duchenne muscular dystrophy; Enzymes; Fibrosis - drug therapy; Fibrosis - metabolism; Fitness equipment; Free radicals; Functional foods & nutraceuticals; Health aspects; Mice; Mice, Inbred C57BL; Mitochondria; Muscle, Skeletal - drug effects; Muscle, Skeletal - metabolism; Muscular dystrophy; Muscular Dystrophy, Animal - drug therapy; Muscular Dystrophy, Animal - metabolism; Muscular Dystrophy, Duchenne - drug therapy; Muscular Dystrophy, Duchenne - metabolism; Myofibrils - drug effects; Myofibrils - metabolism; NF-kappa B - metabolism; Nitric Oxide - metabolism; Oxidative stress; Pathology; Physiological aspects; Polyphenols; Polyphenols - pharmacology; Rodents; Software; Studies; Variance analysis; Vitamin E
• ISSN: 2314-6133; 2314-6141
• DOI: 10.1155/2015/680615

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 terms of muscular architecture and functionality. We observed a reduction of muscle fibrosis deposition and myofiber necrosis together with an amelioration of vascularization. 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 coadjuvate the treatment of DMD.