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Background  Duchenne 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 have important clinical merit for DMD and related neuromuscular diseases. We investigated the therapeutic potential for tranilast, an orally bioavailable anti-allergic agent, to prevent fibrosis in skeletal muscles of  mdx  dystrophic mice.    Results  Three-week-old C57Bl/10 and  mdx  mice received tranilast (~300 mg/kg) in their food for 9 weeks, after which fibrosis was assessed through histological analyses, and functional properties of tibialis anterior muscles were assessed  in situ  and diaphragm muscle strips  in vitro . Tranilast administration did not significantly alter the mass of any muscles in control or  mdx  mice, but it decreased fibrosis in the severely affected diaphragm muscle by 31% compared with untreated  mdx  mice ( P  < 0.05). A similar trend of decreased fibrosis was observed in the tibialis anterior muscles of  mdx  mice ( P  = 0.10). These reductions in fibrotic deposition were not associated with improvements in maximum force-producing capacity, but we did observe small but significant improvements in the resistance to fatigue in both the diaphragm and TA muscles of  mdx  mice treated with tranilast.    Conclusion  Together these findings demonstrate that administration of potent antifibrotic compounds such as tranilast could help preserve skeletal muscle structure, which could ultimately increase the efficacy of pharmacological, cell and gene replacement/correction therapies for muscular dystrophy and related disorders.

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