Animal models for inherited muscle diseases

Abstract

Animal models of inherited muscle diseases are critical to our understanding of the pathophysiological mechanisms of these diseases and serve as surrogates in which approaches to therapy may be evaluated. Many models have been described, including those that have spontaneously occurred as well as models derived from mutagenesis and screening. This chapter focuses primarily on two species, the mouse and the dog, in which the majority of these models have been elucidated. However, inherited muscle disease is described in other species as well. For many of these models the nature of the mutation is known and the features of the disease are presented. Models of dystrophinopathy, limb-girdle muscular dystrophy, merosin deficiency, centronuclear myopathies as well as several other muscle diseases are discussed. In addition, information about husbandry and management of colonies of the animal models is presented.