Skeletal Muscle Fibrosis in the mdx/utrn+/- Mouse Validates Its Suitability as a Murine Model of Duchenne Muscular Dystrophy

摘要: Various therapeutic approaches have been studied for the treatment of Duchenne muscular dystrophy (DMD), but none these led to significant long-term effects in patients. One reason this observed inefficacy may be use inappropriate animal models testing agents. The mdx mouse is most widely used murine model DMD, yet it does not fibrotic progression Other DMD are available that lack one or both alleles utrophin, a functional analog dystrophin. aim study was compare fibrosis and myofiber damage mdx, mdx/utrn+/- double knockout (dko) models. We Masson’s trichrome stain percentage centrally-nucleated myofibers as indicators regeneration, respectively, assess disease diaphragm gastrocnemius muscles harvested from young aged wild-type, dko mice. Our results indicated eight week-old hind limb developed whereas age-matched muscle did (p = 0.002). amount collagen found significantly higher than corresponding wild-type animals, animals 0.0003). Aged mice compared controls 0.003). Mdx well 0.0235), fibrotic. measure difference staining between muscles. support previous reports moderately-affected better we show here apparent by 2 months age.