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Dewulf, Melissa; Köster, Darius Vasco; Sinha, Bidisha; Viaris de Lesegno, Christine; Chambon, Valérie; Bigot, Anne; Bensalah, Mona; Negroni, Elisa; Tardif, Nicolas; Podkalicka, Joanna; Johannes, Ludger; Nassoy, Pierre; Butler-Browne, Gillian; Lamaze, Christophe; Blouin, Cedric M.

Dystrophy-associated caveolin-3 mutations reveal that caveolae couple IL6/STAT3 signaling with mechanosensing in human muscle cells

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  • Medientyp: E-Artikel
  • Titel: Dystrophy-associated caveolin-3 mutations reveal that caveolae couple IL6/STAT3 signaling with mechanosensing in human muscle cells
  • Beteiligte: Dewulf, Melissa; Köster, Darius Vasco; Sinha, Bidisha; Viaris de Lesegno, Christine; Chambon, Valérie; Bigot, Anne; Bensalah, Mona; Negroni, Elisa; Tardif, Nicolas; Podkalicka, Joanna; Johannes, Ludger; Nassoy, Pierre; Butler-Browne, Gillian; Lamaze, Christophe; Blouin, Cedric M.
  • Erschienen: Springer Science and Business Media LLC, 2019
  • Erschienen in: Nature Communications
  • Sprache: Englisch
  • DOI: 10.1038/s41467-019-09405-5
  • ISSN: 2041-1723
  • Schlagwörter: General Physics and Astronomy ; General Biochemistry, Genetics and Molecular Biology ; General Chemistry ; Multidisciplinary
  • Entstehung:
  • Anmerkungen:
  • Beschreibung: <jats:title>Abstract</jats:title><jats:p>Caveolin-3 is the major structural protein of caveolae in muscle. Mutations in the <jats:italic>CAV3</jats:italic> gene cause different types of myopathies with altered membrane integrity and repair, expression of muscle proteins, and regulation of signaling pathways. We show here that myotubes from patients bearing the <jats:italic>CAV3</jats:italic> P28L and R26Q mutations present a dramatic decrease of caveolae at the plasma membrane, resulting in abnormal response to mechanical stress. Mutant myotubes are unable to buffer the increase in membrane tension induced by mechanical stress. This results in impaired regulation of the IL6/STAT3 signaling pathway leading to its constitutive hyperactivation and increased expression of muscle genes. These defects are fully reversed by reassembling functional caveolae through expression of caveolin-3. Our study reveals that under mechanical stress the regulation of mechanoprotection by caveolae is directly coupled with the regulation of IL6/STAT3 signaling in muscle cells and that this regulation is absent in Cav3-associated dystrophic patients.</jats:p>
  • Zugangsstatus: Freier Zugang