title: Defective growth in vitro of Duchenne Muscular Dystrophy myoblasts: the molecular and biochemical basis.
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.
authors.affiliation: 2nd Division of Neurology, 2nd University of Naples School of Medicine, Naples, Italy. mmelone@unina.it
authors.forename: M A
authors.initials: MA
authors.lastname: Melone
authors.forename: G
authors.initials: G
authors.lastname: Peluso
authors.forename: O
authors.initials: O
authors.lastname: Petillo
authors.forename: U
authors.initials: U
authors.lastname: Galderisi
authors.forename: R
authors.initials: R
authors.lastname: Cotrufo
mesh_terms: 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
chemical_list: D017077:Culture Media, Conditioned; D016212:Transforming Growth Factor beta