Defective growth in vitro of Duchenne muscular dystrophy myoblasts: The molecular and biochemical basis

✍ Scribed by Mariarosa A.B. Melone; Gianfranco Peluso; Orsolina Petillo; Umberto Galderisi; Roberto Cotrufo

• Publisher: John Wiley and Sons
• Year: 2000
• Tongue: English
• Weight: 234 KB
• Volume: 76
• Category: Article
• ISSN: 0730-2312
• DOI: 10.1002/(sici)1097-4644(20000101)76:1<118::aid-jcb12>3.0.co;2-f

✦ Synopsis

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-␤1-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-␤1 increase in conditioned media of DMD myoblasts, able to control the myoblast growth by reducing fusion and differentiation of DMD satellite cells.