Defective growth in vitro of Duchenne Muscular Dystrophy myoblasts: the molecular and biochemical basis

J Cell Biochem. 1999 Nov;76(1):118-32. doi: 10.1002/(sici)1097-4644(20000101)76:1<118::aid-jcb12>3.3.co;2-6.

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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Cell Differentiation / physiology
  • Cell Division / physiology*
  • Cell Fusion / physiology
  • Child, Preschool
  • Culture Media, Conditioned
  • Humans
  • Muscle, Skeletal / physiopathology
  • Muscular Dystrophy, Duchenne / pathology*
  • Muscular Dystrophy, Duchenne / physiopathology
  • Regeneration
  • Transforming Growth Factor beta / physiology

Substances

  • Culture Media, Conditioned
  • Transforming Growth Factor beta