Physiological Assessment of Muscle, Heart, and Whole Body Function in the Canine Model of Duchenne Muscular Dystrophy.

Autor: Hakim CH; Department of Molecular Microbiology and Immunology, School of Medicine, The University of Missouri, Columbia, MO, USA., Teixeira J; Department of Molecular Microbiology and Immunology, School of Medicine, The University of Missouri, Columbia, MO, USA., Leach SB; Department of Veterinary Medicine and Surgery, College of Veterinary Medicine, The University of Missouri, Columbia, MO, USA., Duan D; Department of Molecular Microbiology and Immunology, School of Medicine, The University of Missouri, Columbia, MO, USA. duand@missouri.edu.; Department of Biomedical, Biological & Chemical Engineering, College of Engineering, The University of Missouri, Columbia, MO, USA. duand@missouri.edu.; Department of Neurology, School of Medicine, The University of Missouri, Columbia, MO, USA. duand@missouri.edu.; Department of Biomedical Sciences, College of Veterinary Medicine, The University of Missouri, Columbia, MO, USA. duand@missouri.edu.
Jazyk: angličtina
Zdroj: Methods in molecular biology (Clifton, N.J.) [Methods Mol Biol] 2023; Vol. 2587, pp. 67-103.
DOI: 10.1007/978-1-0716-2772-3_5
Abstrakt: 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.
(© 2023. The Author(s), under exclusive license to Springer Science+Business Media, LLC, part of Springer Nature.)
Databáze: MEDLINE