Dystrophin mutations predict cellular susceptibility to oxidative stress

Disatnik, Marie‐Hélène; Chamberlain, Jeffrey S.; Rando, Thomas A.

doi:10.1002/(sici)1097-4598(200005)23:5<784::aid-mus17>3.0.co;2-y

Cited by 59 publications

(31 citation statements)

References 59 publications

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“…Furthermore, mdx muscle cells and DMD skin fibroblasts show an increased susceptibility to oxidative damage (27,28), suggesting a role of the glutathione antioxidant system in dystrophic pathology (28). Recently, increased GSSG/GSH was reported in young (prenecrotic) mdx hindlimb muscle, suggesting an abnormal glutathione status in dystrophic muscle under basal conditions and before muscle necrosis (2).…”

Section: Discussionmentioning

confidence: 99%

L-Glutamine Administration Reduces Oxidized Glutathione and MAP Kinase Signaling in Dystrophic Muscle of mdx Mice

et al. 2008

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ABSTRACT:To determine whether glutamine (Gln) reduces the ratio of oxidized to total glutathione (GSSG/GSH) and extracellular signal-regulated kinase (ERK1/2) activation in dystrophic muscle. Four-week old mdx mice, an animal model for Duchenne muscular dystrophy and control (C57BL/10) received daily intraperitoneal injections of L-Gln (500 mg/kg/d) or 0.9% NaCl for 3 d. GSH and GSSG concentrations in gastrocnemius were measured using a standard enzymatic recycling procedure. Free amino acid concentrations in gastrocnemius were determined by ion exchange chromatography. Phosphorylated protein levels of ERK1/2 in quadriceps were examined using Western Blot. L-Gln decreased GSSG and GSSG/GSH (an indicator of oxidative stress). This was associated with decreased ERK1/2 phosphorylation. Muscle free Gln, glutamate (Glu), and the sum (Gln ϩ Glu) were higher in mdx versus C57BL/10, at the basal level. Exogenous Gln decreased muscle free Glu and Gln ϩ Glu in mdx only, whereas Gln was not affected. In conclusion, exogenous Gln reduces GSSG/GSH and ERK1/2 activation in dystrophic skeletal muscle of young mdx mice, which is associated with decreased muscle free Glu and Gln ϩ Glu. This antioxidant protective mechanism provides a molecular basis for Gln's antiproteolytic effect in Duchenne muscular dystrophy children.

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Section: Discussionmentioning

confidence: 99%

L-Glutamine Administration Reduces Oxidized Glutathione and MAP Kinase Signaling in Dystrophic Muscle of mdx Mice

et al. 2008

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“…ba Stand for increase and decrease, respectively. susceptibility of MuSC-derived myotubes to oxidative stress is associated with the severity of the dystrophy (assessed by the expression of different lengths of the dystrophin protein), and the extent of protein oxidation, suggesting that the DCG complex has important functions in antioxidant defense (88). It was shown that nNOS, which binds to DGC, has no impact on MuSC susceptibility to oxidative stress (363).…”

Section: Muscular Dystrophiesmentioning

confidence: 99%

Redox Control of Skeletal Muscle Regeneration

Moal

Pialoux

Juban

et al. 2017

Antioxidants & Redox Signaling

148

120

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Skeletal muscle shows high plasticity in response to external demand. Moreover, adult skeletal muscle is capable of complete regeneration after injury, due to the properties of muscle stem cells (MuSCs), the satellite cells, which follow a tightly regulated myogenic program to generate both new myofibers and new MuSCs for further needs. Although reactive oxygen species (ROS) and reactive nitrogen species (RNS) have long been associated with skeletal muscle physiology, their implication in the cell and molecular processes at work during muscle regeneration is more recent. This review focuses on redox regulation during skeletal muscle regeneration. An overview of the basics of ROS/RNS and antioxidant chemistry and biology occurring in skeletal muscle is first provided. Then, the comprehensive knowledge on redox regulation of MuSCs and their surrounding cell partners (macrophages, endothelial cells) during skeletal muscle regeneration is presented in normal muscle and in specific physiological (exercise-induced muscle damage, aging) and pathological (muscular dystrophies) contexts. Recent advances in the comprehension of these processes has led to the development of therapeutic assays using antioxidant supplementation, which result in inconsistent efficiency, underlying the need for new tools that are aimed at precisely deciphering and targeting ROS networks. This review should provide an overall insight of the redox regulation of skeletal muscle regeneration while highlighting the limits of the use of nonspecific antioxidants to improve muscle function. Antioxid. Redox Signal. 27, 276-310.

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“…Indeed, cytokines that inhibit satellite cell proliferation or promote fibrosis, such as transforming growth factor-␤ (5) and others, have been found to be upregulated in dystrophin-deficient muscles in vivo. Other characteristics associated with dystrophin deficiency, such as dysregulation of skeletal muscle blood flow during muscle activation (49), a differential sensitivity to free radicals (9), and alterations in mechanotransduction cell signaling mechanisms (24), could also be involved in the accelerated dystrophic pathology of the mdx diaphragm. Exposure of the diaphragm to notexin, which, in addition to destroying mature myofibers also has marked effects on proinflammatory mediators (17) and the extracellular matrix environment (22), may also transiently affect one or more of these factors.…”

Section: Implications For Pathophysiology Of Dmdmentioning

confidence: 99%