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 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.
Highlights
Muscular dystrophies (MDs) are a heterogeneous group of disorders characterized by muscular wasting and inflammation that cause reduction of force and premature death [1]
Duchenne muscular dystrophy (DMD), the most common form of muscular dystrophy, is characterized by muscular wasting caused by dystrophin deficiency that ends in force reduction and premature death
We found that the distribution curves of treated mice shifted to the right in comparison to that one related to mdx control group, proving that there was a significant increase in fiber cross-section fiber area (CSA) in both muscles examined (TA and QA muscles) (F test to compare variance was significant for P < 0,0001) (Figures 1(b)–1(d))
Summary
Muscular dystrophies (MDs) are a heterogeneous group of disorders characterized by muscular wasting and inflammation that cause reduction of force and premature death [1]. The role of inflammatory cells is not completely understood but it is known that they could exacerbate muscular wasting, either directly or by secreting mediators, like cytokines and complement’s component [2], and by reactive oxygen species (ROS) production [3,4,5]. Different groups demonstrated that increased ROS production was a primary feature of dystrophic muscle damage and not a consequence of muscular degeneration [7, 8]. Others showed that ROS-mediated effect on dystrophic muscles increased protein oxidation, which could cause a wide range of deleterious effects on muscle contractile function [9], both in mdx mice [10] and in DMD patients [11]. Even if the precise mechanism by which oxidative stress causes mitochondria dysfunction is not clear, numerous studies reported that ROS could negatively modify both expression and structural conformation of the proteins that are involved in normal mitochondria functions [13, 14]
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