Critical Assessment of the mdx Mouse with Ex Vivo Eccentric Contraction of the Diaphragm Muscle

Muscle function and health progressively deteriorate during the progression of muscle dystrophies. The ability to objectively characterize muscle function and muscle damage is useful not only when comparing variants of dystrophy models, but also for characterizing the effects of interventions aiming to improve or halt the progressive decline of muscle function and muscle health. The protocols in this chapter describe the use of ex vivo eccentric contraction of the diaphragm muscle as a measure of muscle susceptibility to damage. Because muscle has a robust regenerative capacity, unhealthy muscle may be functionally close to normal; therefore, protocols for ex vivo characterization of muscle are often essential for assessing the effects of interventions. Additional methods that can be applied for assessment of dystrophic muscle are also highlighted. © 2018 by John Wiley & Sons, Inc.