Protocol for in vivo analysis of muscle function in porcine models for muscular dystrophies.

Abstract

Porcine models carrying DMD mutations recapitulate Duchenne and Becker muscular dystrophy (DMD and BMD), inherited disorders leading to progressive muscle weakness, with DMD presenting a far more severe and rapidly progressive phenotype. Accurate in vivo muscle function assessment is essential for monitoring disease progression and as an efficacy readout for therapeutic intervention. We present a protocol to evaluate muscle strength, dynamics, and fatigue in dystrophic pigs, including design setup, anesthesia, measurement, and data analysis. For complete details on the use and execution of this protocol, please refer to Blasi et al..