Quel avenir pour la souris mdx comme modèle de la dystrophie musculaire de Duchenne ?

Abstract

Duchenne muscular dystrophy (DMD) is a severe form of an X-linked muscular dystrophy characterized by a progressive weakness of the striated muscles. Many animal models of DMD have been extensively studied for the last decades. Research which have been carried out on these models have substantially contributed to a better understanding of muscular pathology. The recent discovery of DMD gene and identification of its protein product, the dystrophin, brought a new insight in the molecular mechanisms which are involved in the disease. In addition, DMD gene characterization provides a new molecular tool for genetically based classification of mutant animal models. The present review particularly emphasizes the interest of one of these models, the mdx mouse. The main histopathological features of this animal model are shared in common with the other dystrophinopathies known today. The interest of mdx mouse in view of new therapeutical approaches is considered.