Patients with dystrophinopathy show evidence of increased oxidative stress

Rodriguez, Marisela; Tarnopolsky, Mark A.

doi:10.1016/s0891-5849(03)00141-2

Cited by 103 publications

(78 citation statements)

References 12 publications

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“…Oxidative stress induced by either environmental agents or endogenous biochemical reactions has been implicated in the pathogenesis of a variety of degenerative and proliferative diseases. [21][22][23] As a reliable biomarker of oxidative DNA damage, higher levels of 8-OHdG have been observed in the lungs of cigarette smokers, 24 the liver of patients with chronic hepatitis, 25 the breast tissue of benign and malignant tumors, 26 the trabecular meshwork of glaucoma patients, 27 the renalcell carcinoma, 28 the urine of patients with dystrophinopathy, 29 the leucocytes of patients with Leber's hereditary optic neuropathy 30 and patients on chronic hemodialysis, 31 and heart mitochondrial DNA of patients with atrial fibrillation. 32 To our knowledge, this is the first study to explore the increase of 8-OHdG content in pterygium tissue.…”

Section: Discussionmentioning

confidence: 99%

Increased oxidative DNA damage, 8-hydroxydeoxy- guanosine, in human pterygium

Kau

Tsai

Lee³

et al. 2005

Eye

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Section: Discussionmentioning

confidence: 99%

Increased oxidative DNA damage, 8-hydroxydeoxy- guanosine, in human pterygium

Kau

Tsai

Lee³

et al. 2005

Eye

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“…9 -11 Markers of oxidative stress have been detected in muscles of either DMD patients or mdx mice. 9,12,13 An involvement of reactive oxygen intermediates is also supported by observations of increased biological by-products of oxidative stress, 14 reduced cellular antioxidants (glutathione and vitamin E), and altered concentrations of antioxidant enzymes. 12,15 Recently, the role of nuclear factor-B (NF-B) in the skeletal muscle-wasting process is gaining increasing attention, mainly because NF-B is activated in response to several inflammatory molecules that cause muscle loss.…”

mentioning

confidence: 87%

Lipid Peroxidation Inhibition Blunts Nuclear Factor-κB Activation, Reduces Skeletal Muscle Degeneration, and Enhances Muscle Function in mdx Mice

Messina

Altavilla

Aguennouz

et al. 2006

The American Journal of Pathology

107

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Duchenne muscular dystrophy (DMD) is a progressive muscle-wasting disease resulting from lack of the sarcolemmal protein dystrophin. However, the mechanism leading to the final disease status is not fully understood. Several lines of evidence suggest a role for nuclear factor (NF)-B in muscle degeneration as well as regeneration in DMD patients and mdx mice. We investigated the effects of blocking NF-B by inhibition of oxidative stress/lipid peroxidation on the dystrophic process in mdx mice. Five-week-old mdx mice received three times a week for 5 weeks either IRFI-042 (20 mg/kg), a strong antioxidant and lipid peroxidation inhibitor, or its vehicle. IRFI-042 treatment increased forelimb strength (؉22%, P < 0.05) and strength normalized to weight (؉23%, P < 0.05) and decreased fatigue (؊45%, P < 0.05). It also reduced serum creatine kinase levels (P < 0.01) and reduced muscle-conjugated diene content and augmented muscle-reduced glutathione (P < 0.01). IRFI-042 blunted NF-B DNA-binding activity and tumor necrosis factor-␣ expression in the dystrophic muscles (P < 0.01), reducing muscle necrosis (P < 0.01) and enhancing regeneration (P < 0.05). Our data suggest that oxidative stress/lipid peroxidation represents one of the mechanisms activating NF-B and the consequent pathogenetic cascade in mdx muscles.

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“…Because oxidative damage is found in the muscle even before the onset of muscle necrosis (Disatnik et al, 1998), reactive oxygen species (ROS) have been implicated in the pathogenesis of DMD (Rodriguez and Tarnopolsky, 2003). Administration of the antioxidant N-acetyl cysteine to mdx mice decreases ROS levels, significantly ameliorates stretchinduced muscle damage, and reduces the number of centrally nucleated premature muscle fibers (Whitehead et al, 2008).…”

Section: Introductionmentioning

confidence: 99%

Resveratrol Ameliorates Muscular Pathology in the Dystrophic mdx Mouse, a Model for Duchenne Muscular Dystrophy

Hori¹,

Kuno²,

Hosoda³

et al. 2011

J Pharmacol Exp Ther

151

173

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Muscular dystrophies are inherited myogenic disorders accompanied by progressive skeletal muscle weakness and degeneration. We previously showed that resveratrol (3,5,4Ј-trihydroxy-trans-stilbene), an antioxidant and activator of the NAD ϩ -dependent protein deacetylase SIRT1, delays the progression of heart failure and prolongs the lifespan of ␦-sarcoglycan-deficient hamsters. Because a defect of dystroglycan complex causes muscular dystrophies, and ␦-sarcoglycan is a component of this complex, we hypothesized that resveratrol might be a new therapeutic tool for muscular dystrophies. Here, we examined resveratrol's effect in mdx mice, an animal model of Duchenne muscular dystrophy. mdx mice that received resveratrol in the diet for 32 weeks (4 g/kg diet) showed significantly less muscle mass loss and nonmuscle interstitial tissue in the biceps femoris compared with mdx mice fed a control diet. In the muscles of these mice, resveratrol significantly decreased oxidative damage shown by the immunostaining of nitrotyrosine and 8-hydroxy-2Ј-deoxyguanosine and suppressed the up-regulation of NADPH oxidase subunits Nox4, Duox1, and p47 phox . Resveratrol also reduced the number of ␣-smooth muscle actin (␣-SMA) ϩ myofibroblast cells and endomysial fibrosis in the biceps femoris, although the infiltration of CD45ϩ inflammatory cells and increase in transforming growth factor-␤1 (TGF-␤1) were still observed. In C2C12 myoblast cells, resveratrol pretreatment suppressed the TGF-␤1-induced increase in reactive oxygen species, fibronectin production, and expression of ␣-SMA, and SIRT1 knockdown blocked these inhibitory effects. SIRT1 small interfering RNA also increased the expression of Nox4, p47 phox , and ␣-SMA in C2C12 cells. Taken together, these findings indicate that SIRT1 activation may be a useful strategy for treating muscular dystrophies.

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Patients with dystrophinopathy show evidence of increased oxidative stress

Cited by 103 publications

References 12 publications

Increased oxidative DNA damage, 8-hydroxydeoxy- guanosine, in human pterygium

Increased oxidative DNA damage, 8-hydroxydeoxy- guanosine, in human pterygium

Lipid Peroxidation Inhibition Blunts Nuclear Factor-κB Activation, Reduces Skeletal Muscle Degeneration, and Enhances Muscle Function in mdx Mice

Resveratrol Ameliorates Muscular Pathology in the Dystrophic mdx Mouse, a Model for Duchenne Muscular Dystrophy

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