Animal models of muscular dystrophy

Abstract

Muscular dystrophy is a severe degenerative disorder of the skeletal muscle, characterized by progressive muscle weakness. One subgroup of this disease is caused by a defect in the genes encoding the components of the dystrophin-glycoprotein complex. Such a defect results in a significant disruption of membrane integrity and/or stability and, consequently, a sustained increase in cytosolic Ca2+ concentration ([Ca2+] i). Abnormal Ca2+ homeostasis, especially under mechanical stress, is believed to be a key molecular event in the pathology of muscular dysgenesis. In this chapter, we will review the animal models of muscular dystrophy useful for understanding the pathophysiology of the disease. Particularly, we will focus on stretch-activated TRP channels, which were reported to have critical pathological significance, and discuss the therapeutic potential of these channels for muscle dystrophy. We will also briefly summarize in vivo and in vitro procedures using dystrophic animal models, isolated muscle fibers, and cultured myotubes. © 2012 Springer Science+Business Media, LLC.