The role of reactive oxygen species in the hearts of dystrophin-deficient mdx mice.

@article{Williams2007TheRO,
  title={The role of reactive oxygen species in the hearts of dystrophin-deficient mdx mice.},
  author={Iwan A. Williams and David G. Allen},
  journal={American journal of physiology. Heart and circulatory physiology},
  year={2007},
  volume={293 3},
  pages={
          H1969-77
        }
}
  • Iwan A. Williams, D. Allen
  • Published 1 September 2007
  • Biology, Medicine
  • American journal of physiology. Heart and circulatory physiology
Duchenne muscular dystrophy (DMD) is caused by deficiency of the cytoskeletal protein dystrophin. Oxidative stress is thought to contribute to the skeletal muscle damage in DMD; however, little is known about the role of oxidative damage in the pathogenesis of the heart failure that occurs in DMD patients. The dystrophin-deficient (mdx) mouse is an animal model of DMD that also lacks dystrophin. The current study investigates the role of the antioxidant N-acetylcysteine (NAC) on mdx… 
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144 Citations
Highly Influential Citations
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Background Citations
67
Methods Citations
6
Results Citations
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144 Citations

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Citation Type

Delayed cardiomyopathy in dystrophin deficient mdx mice relies on intrinsic glutathione resource.
TLDR
Low glutathione resource hastens the onset of cardiomyopathy linked to a defect in dystrophin in mdx mice, relevant to the glutathionine deficiency that DMD patients may suffer.
N‐Acetylcysteine ameliorates skeletal muscle pathophysiology in mdx mice
TLDR
The findings suggest that ROS play an important role in the dystrophic pathogenesis, both in terms of activating damage pathways and in regulating the expression of some dystrophin‐associated membrane proteins.
Dystrophin Is Required for the Normal Function of the Cardio-Protective KATP Channel in Cardiomyocytes
TLDR
It is shown that the mdx mouse heart has defects consistent with alteration in compounds that regulate energy homeostasis including a marked decrease in creatine-phosphate (PC), and hypothesize that dystrophin acts as a scaffolding protein organizing the KATP channel complex and the enzymes necessary for its correct functioning.
Normalization of connexin 43 protein levels prevents cellular and functional signs of dystrophic cardiomyopathy in mice
Metabolic Alterations in Cardiomyocytes of Patients with Duchenne and Becker Muscular Dystrophies
TLDR
The current literature featuring the metabolic alterations observed in the dystrophic heart of the mdx mouse is reviewed, i.e., the best-studied animal model of the disease, and their pathophysiological role in the DMD heart is discussed.
Acute AT1R blockade prevents isoproterenol-induced injury in mdx hearts.
Diaphragm tension reduced in dystrophic mice by an oxidant, hypochlorous acid.
TLDR
The results showed that HOCl, at micromolar or millimolar concentrations, can modify sarcoplasmic reticulum Ca2+ uptake and that this effect was more pronounced in diaphragm muscle from mdx mice.
Contribution of oxidative stress to pathology in diaphragm and limb muscles with Duchenne muscular dystrophy
TLDR
The pathobiology of dystrophin deficiency in diaphragm and limb muscle primarily in mouse models is focused upon, with a rationale for development of targeted therapeutic antioxidants in DMD patients.
Hierarchical accumulation of RyR post-translational modifications drives disease progression in dystrophic cardiomyopathy.
TLDR
The findings suggest that increased RyR Ca(2+) sensitivity precedes and presumably drives the progression of dystrophic cardiomyopathy, with oxidative stress initiating its development.
Amplification of proinflammatory phenotype, damage, and weakness by oxidative stress in the diaphragm muscle of mdx mice.
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References

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TLDR
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TLDR
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TLDR
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TLDR
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TLDR
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TLDR
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TLDR
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