期刊
FEBS LETTERS
卷 581, 期 26, 页码 5099-5104出版社
WILEY
DOI: 10.1016/j.febslet.2007.09.055
关键词
caveolin 3; dystrophy; AKT; follistatin; NFATC2; IL-4; murf-1; atrogin
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 Delta TFT respectively, which cause the limb-girdle muscular dystrophy I-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 Delta TFT 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. (C) 2007 Federation of European Biochemical Societies. Published by Elsevier B.V. All rights reserved.
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