Molecular, cellular, and muscle strip mechanics of the mdx mouse diaphragm

• Published in: American Journal of Physiology: Cell Physiology Vol. 304; no. 9; pp. C873 - C880
• Main Authors: Bates, Genevieve, Sigurdardottir, Sara, Kachmar, Linda, Zitouni, Nedjma B, Benedetti, Andrea, Petrof, Basil J, Rassier, Dilson, Lauzon, Anne-Marie
• Format: Journal Article
• Language: English
• Published: United States American Physiological Society 01.05.2013
• Subjects: Animals; Biomechanical Phenomena; Cells; Diaphragm - physiopathology; In Vitro Techniques; Isometric Contraction; Kinetics; Male; Mice; Mice, Inbred C57BL; Mice, Inbred mdx; Molecules; Muscle Fibers, Skeletal - physiology; Muscular Dystrophy, Duchenne - metabolism; Muscular Dystrophy, Duchenne - pathology; Muscular Dystrophy, Duchenne - physiopathology; Musculoskeletal system; Myosins - chemistry; Myosins - physiology; Proteolysis; Rodents; duchenne muscular dystrophy; in vitro motility assay; dystrophin

Duchenne muscular dystrophy (DMD) is a lethal disorder caused by defects in the dystrophin gene, which leads to respiratory or cardiac muscle failure. Lack of dystrophin predisposes the muscle cell sarcolemmal membrane to mechanical damage. However, the role of myosin in this muscle weakness has bee...