Phenotypic behavior of C2C12 myoblasts upon expression of the dystrophy-related caveolin-3 P104L and TFT mutants |
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Authors: | Fanzani Alessandro Stoppani Elena Gualandi Laura Giuliani Roberta Galbiati Ferruccio Rossi Stefania Fra Anna Preti Augusto Marchesini Sergio |
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Institution: | Department of Biomedical Sciences and Biotechnology, Unit of Biochemistry, University of Brescia, Viale Europa 11, 25123 Brescia, Italy. fanzani@med.unibs.it |
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Abstract: | Caveolin-3 (Cav-3) is the main scaffolding protein present in myofiber caveolae. We transfected C2C12 myoblasts with dominant negative forms of Cav-3, P104L or DeltaTFT, respectively, which cause the limb-girdle muscular dystrophy 1-C. Both these forms triggered Cav-3 loss during C2C12 cell differentiation. The P104L mutation reduced myofiber formation by impaired AKT signalling, accompanied by dramatic expression of the E3 ubiquitin ligase Atrogin. On the other hand, the DeltaTFT mutation triggered hypertrophic myotubes sustained by prolonged AKT activation, but independent of increased levels of follistatin and interleukin 4 expression. These data suggest that separated mutations within the same dystrophy-related gene may cause muscle degeneration through different mechanisms. |
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Keywords: | DMEM Dulbecco’s modified Eagle’s medium FBS fetal bovine serum HS horse serum MHC myosin heavy chain NFATC nuclear factor of activated T-cells IL-4 Interleukin 4 |
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