ANALYSIS OF MYOGENIC MARKERS IN DUCHENNE MUSCULAR DYSTROPHY CELL MODELS

Abstract

The goal of this study is to compare the in vitro differentiation potential of Duchenne Muscular Dystrophy (DMD) and non-diseased patient-derived skeletal muscle myoblasts during myogenesis. The differentiation and fusion of myoblasts into multinucleate myotubes and the maturity of these myotubes was assessed based on morphology, immunohistochemistry (IHC) analysis of myotubes, as well as transcript profiles of myogenic markers. Human skeletal muscle myoblasts derived from three non-diseased and three DMD human patients were evaluated in multiple time course studies. Morphological evaluation as well as IHC analysis indicated that the DMD patient-derived myoblasts have diminished capacity to differentiate and form mature myotubes. Gene expression profiling also revealed significantly reduced basal transcript levels of myogenic markers in DMD patient-derived cells as well as the impaired induction of these transcripts during differentiation.