Defective growth in vitro of Duchenne Muscular Dystrophy myoblasts: the molecular and biochemical basis |
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Authors: | Melone M A Peluso G Petillo O Galderisi U Cotrufo R |
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Institution: | 2nd Division of Neurology, 2nd University of Naples School of Medicine, Naples, Italy. mmelone@unina.it |
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Abstract: | As the molecular basis of Duchenne Muscular Dystrophy (DMD) was being discovered, increasing focus was placed on the mechanisms of progressive failure of myoregeneration. In this study, we propose a pathogenesis model for DMD, where an autocrine growth factor release of TGF-beta1-from necrotic myofibers-could contribute to the increasing loss of muscle regeneration. In fact, we report evidence that DMD myoblasts reduce their proliferation rate, in time and later cultures; in connection with this, we observed TGF-beta1 increase in conditioned media of DMD myoblasts, able to control the myoblast growth by reducing fusion and differentiation of DMD satellite cells. |
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