Defective growth in vitro of Duchenne Muscular Dystrophy myoblasts: the molecular and biochemical basis.
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.
Melone
M A
MA
2nd Division of Neurology, 2nd University of Naples School of Medicine, Naples, Italy. mmelone@unina.it
Peluso
G
G
Petillo
O
O
Galderisi
U
U
Cotrufo
R
R
D002454:Cell Differentiation / Q000502:physiology; D002455:Cell Division / Q000502:physiology*; D002459:Cell Fusion / Q000502:physiology; D002675:Child, Preschool; D017077:Culture Media, Conditioned; D006801:Humans; D018482:Muscle, Skeletal / Q000503:physiopathology; D020388:Muscular Dystrophy, Duchenne / Q000473:pathology* / Q000503:physiopathology; D012038:Regeneration; D016212:Transforming Growth Factor beta / Q000502:physiology
D017077:Culture Media, Conditioned; D016212:Transforming Growth Factor beta