The force-temperature relationship in healthy and dystrophic mouse diaphragm; implications for translational study design.

In the field of muscular dystrophy, striated muscle function is often assessed in vitro in dystrophin-deficient mdx mice in order to test the impact of a potential treatment strategy. Although many past studies have assessed diaphragm contractile function at or near room temperature, the diaphragm performs in vivo at 37°C. To improve translation of bench-top results to possible clinical application, we studied temperature-dependence of contractile performance in wild-type (C57BL/10) and mdx muscle strips at temperatures from 25°C to 37°C.

Early treatment with lisinopril and spironolactone preserves cardiac and skeletal muscle in Duchenne muscular dystrophy mice.

Background: Nearly universal cardiomyopathy in Duchenne muscular dystrophy (DMD) contributes to heart failure and death. Because DMD patients show myocardial fibrosis well before functional impairment, we postulated that earlier treatment using drugs with antifibrotic effect may be beneficial.

Methods And Results: Three groups of 10 utrn(+/-);mdx, or "het" mice, deficient for dystrophin and haploinsufficient for utrophin with skeletal myopathy and cardiomyopathy that closely mimics clinical DMD were studied.