Critical Assessment of the mdx Mousewith Ex Vivo Eccentric Contraction of theDiaphragm Muscle

摘要

Muscle function and health progressively deteriorate during the progressionof muscle dystrophies. The ability to objectively characterize muscle functionand muscle damage is useful not only when comparing variants of dystrophymodels, but also for characterizing the effects of interventions aiming to improveor halt the progressive decline of muscle function and muscle health. Theprotocols in this chapter describe the use of ex vivo eccentric contraction of thediaphragm muscle as a measure of muscle susceptibility to damage. Becausemuscle has a robust regenerative capacity, unhealthy muscle may be functionallyclose to normal; therefore, protocols for ex vivo characterization of muscleare often essential for assessing the effects of interventions.Additional methodsthat can be applied for assessment of dystrophic muscle are also highlighted.