Tranilast administration reduces fibrosis and improves fatigue resistance in muscles of mdx dystrophic mice

@article{Swiderski2014TranilastAR,
  title={Tranilast administration reduces fibrosis and improves fatigue resistance in muscles of mdx dystrophic mice},
  author={K. Swiderski and M. Todorov and S. Gehrig and T. Naim and A. Chee and D. Stapleton and R. Koopman and G. Lynch},
  journal={Fibrogenesis \& Tissue Repair},
  year={2014},
  volume={7},
  pages={1 - 1}
}
BackgroundDuchenne muscular dystrophy (DMD) is a severe and progressive muscle-wasting disorder caused by mutations in the dystrophin gene that result in the absence of the membrane-stabilising protein dystrophin. Dystrophic muscle fibres are susceptible to injury and degeneration, and impaired muscle regeneration is associated with fibrotic deposition that limits the efficacy of potential pharmacological, cell- and gene-based therapies. Novel treatments that can prevent or attenuate fibrosis… Expand
Glycine administration attenuates progression of dystrophic pathology in prednisolone-treated dystrophin/utrophin null mice
TLDR
Findings provide strong evidence that glycine supplementation may be a safe, simple and effective adjuvant for improving the efficacy of prednisolone treatment and improving the quality of life for DMD patients. Expand
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TLDR
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TLDR
If calcium channels, reactive oxygen species, and nuclear factor-κB signaling factors are the most promising targets as master regulators of inflammatory response in DMD skeletal muscles, if they are combined with an oligonucleotide-based exon skipping therapy to restore dystrophin expression, the anti-inflammatory drug therapies may address the present therapeutic limitation of low efficiency for DMD. Expand
Some dystrophy phenotypes of dystrophin‐deficient mdx mice are exacerbated by mild, repetitive daily stress
TLDR
The results indicate that the response of mdx mice to an acute mild stress is non‐habituating and that when that stressor is repeated daily for weeks, it is sufficient to exacerbate some phenotypes associated with dystrophinopathy in m dx mice. Expand
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TLDR
The involvement of TRPV2 in a stretch-activated calcium influx pathway in dystrophic cardiomyopathy is established, contributing to the defective cellular Ca2+ handling in this disease. Expand
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TLDR
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Use of EP3533-Enhanced Magnetic Resonance Imaging as a Measure of Disease Progression in Skeletal Muscle of mdx Mice
TLDR
EP3533 can be used over time as an outcome measure to quantify treatment effect of an established anti-fibrotic drug and significant correlation seen between histology and EP3533 signal change is seen. Expand
Muscle-specific deletion of SOCS3 increases the early inflammatory response but does not affect regeneration after myotoxic injury
TLDR
Loss of SOCS3 expression in mature muscle fibers increased the inflammatory response to myotoxic injury but did not impair muscle regeneration in either adult or old mice, suggesting reduced SOCS 3 expression in muscle fibers is unlikely to underlie impaired muscle regeneration. Expand
Disease course in mdx:utrophin+/− mice: comparison of three mouse models of Duchenne muscular dystrophy
TLDR
Mdx:utrophin+/− mice may be a more useful animal model than mdx or dko mice for investigating long‐term efficacy of potential treatments when fibrosis or muscle function is the focus. Expand
Immunophenotype of a Rat Model of Duchenne's Disease and Demonstration of Improved Muscle Strength After Anti-CD45RC Antibody Treatment
TLDR
Anti-CD45RC MAb treatment has potential in the treatment of DMD and might eventually result in reduction or elimination of CS use. Expand
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