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

Article


van Erp, Christel, Irwin, Nicole G. and Hoey, Andrew J.. 2006. "Long-term administration of pirfenidone improves cardiac function in mdx mice." Muscle and Nerve. 34 (3), pp. 327-334. https://doi.org/10.1002/mus.20590
Article Title

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

ERA Journal ID14530
Article CategoryArticle
Authorsvan Erp, Christel (Author), Irwin, Nicole G. (Author) and Hoey, Andrew J. (Author)
Journal TitleMuscle and Nerve
Journal Citation34 (3), pp. 327-334
Number of Pages8
Year2006
Place of PublicationHoboken, NJ. United States
ISSN0148-639X
1097-4598
Digital Object Identifier (DOI)https://doi.org/10.1002/mus.20590
Web Address (URL)https://onlinelibrary.wiley.com/doi/full/10.1002/mus.20590?casa_token=fVm5GVnQ6qAAAAAA%3ABa8GaH5Dt_yjcLSj2R9qlaqkPqJWVxKRvozezhM_06cwvUplRYyTj7WvpWQ6USYu1T2UYdfLR3IOYw
Abstract

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 the cytokine, TGF-β, is a likely mediator. This study tested whether the TGF-β antagonist, pirfenidone, could reduce cardiac fibrosis. Eight-month-old mdx mice were treated for 7 months with 0.4%, 0.8%, and 1.2% of pirfenidone in drinking water: untreated water was given to control mdx and C57 mice. Mice treated with 0.8% and 1.2% pirfendone had lowered cardiac TGF-β mRNA and improved in vitro cardiac contractility (P<0.05) to levels consistent with C57 mice, yet without a change in cardiac stiffness or fibrosis. These results show that the TGF-β antagonist, pirfenidone can improve cardiac function in mdx mice, potentially providing a new avenue for developing cardiac therapies for patients with Duchenne muscular dystrophy.

KeywordsDuchenne muscular dystrophy; fibrosis; mdx mice; pirfenidone; TGF-β
ANZSRC Field of Research 2020310999. Zoology not elsewhere classified
310506. Gene mapping
320101. Cardiology (incl. cardiovascular diseases)
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Byline AffiliationsCentre for Systems Biology
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