Abstract
Duchenne muscular dystrophy (DMD) is a lethal muscle disease caused by dystrophin deficiency. Patients gradually lose motor function, become wheelchair-bound, and die from respiratory and/or cardiac muscle failure. Dystrophin-null dogs have been used as a large animal model for DMD since 1988 and are considered an excellent bridge between rodent models and human patients. While numerous protocols have been published for studying muscle and heart physiology in mice, few such protocols exist for studying skeletal muscle contractility, heart function, and whole-body activity in dogs. Over the last 20 years, we have developed and adapted an array of assays to evaluate whole-body movement, gait, single muscle force, whole limb torque, cardiac electrophysiology, and hemodynamic function in normal and dystrophic dogs. In this chapter, we present detailed working protocols for these assays and lessons we learned during the development and use of these protocols.
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Acknowledgments
The research on the canine DMD model in the Duan lab is currently supported by the National Institutes of Health (NS-90634 and AR-70517), Jesse Davidson Foundation-Defeat Duchenne Canada, Hope for Javier, Jackson Freel DMD Research Fund, Parent Project Muscular Dystrophy, Jett Foundation, Michael’s Cause, Ryan’s Quest, Solid Biosciences Inc., and the University of Missouri.
Disclosure
DD is a member of the scientific advisory board for Solid Biosciences and equity holders of Solid Biosciences. DD is a member of the scientific advisory board for Sardocor Corp. In the last three years, the Duan lab has received research supports unrelated to this project from Solid Biosciences and Edgewise.
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Hakim, C.H., Teixeira, J., Leach, S.B., Duan, D. (2023). Physiological Assessment of Muscle, Heart, and Whole Body Function in the Canine Model of Duchenne Muscular Dystrophy. In: Maruyama, R., Yokota, T. (eds) Muscular Dystrophy Therapeutics. Methods in Molecular Biology, vol 2587. Humana, New York, NY. https://doi.org/10.1007/978-1-0716-2772-3_5
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DOI: https://doi.org/10.1007/978-1-0716-2772-3_5
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