van Erp, Christel and 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. ISSN 0148-639X
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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.
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