Long-term administration of pirfenidone improves cardiac function in mdx mice.

@article{Erp2006LongtermAO,
  title={Long-term administration of pirfenidone improves cardiac function in mdx mice.},
  author={Christel van Erp and Nicole G Irwin and Andrew J Hoey},
  journal={Muscle & nerve},
  year={2006},
  volume={34 3},
  pages={327-34}
}
Duchenne muscular dystrophy, an X-linked recessive neuromuscular disorder due to lack of the protein dystrophin, manifests as progressive muscle degeneration and cardiomyopathy with increased fibrosis. The exact mechanisms involved in fibrosis are unknown, but a cytokine, transforming growth factor-beta (TGF-beta), is a likely mediator. This study tested whether the TGF-beta antagonist, pirfenidone, could reduce cardiac fibrosis. Eight-month-old mdx mice were treated for 7 months with 0.4%, 0.8… CONTINUE READING

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A primer on current progress in cardiac fibrosis.

Canadian journal of physiology and pharmacology • 2017
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