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

Citation
Mab. Melone et al., Defective growth in vitro of Duchenne Muscular Dystrophy myoblasts: The molecular and biochemical basis, J CELL BIOC, 76(1), 2000, pp. 118-132
Citations number
37
Categorie Soggetti
Cell & Developmental Biology
Journal title
JOURNAL OF CELLULAR BIOCHEMISTRY
ISSN journal
07302312 → ACNP
Volume
76
Issue
1
Year of publication
2000
Pages
118 - 132
Database
ISI
SICI code
0730-2312(200001)76:1<118:DGIVOD>2.0.ZU;2-6
Abstract
As the molecular basis of Duchenne Muscular Dystrophy (DMD) was being disco vered, 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-beta 1-from necrotic myof ibers-could contribute to the increasing loss of muscle regeneration. In fa ct, we report evidence that DMD myoblasts reduce their proliferation rate i n time and later cultures; in connection with this, we observed TGF-beta 1 increase in conditioned media of DMD myoblasts, able to control the myoblas t growth by reducing fusion and differentiation of DMD satellite cells. J. Cell. Biochem. 76:118-132, 1999. (C) 1999 Wiley-Liss, Inc.