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EndMT contributes to the onset and progression of cerebral cavernous malformations

Author

Listed:
  • Luigi Maddaluno

    (IFOM Fondazione, FIRC Institute of Molecular Oncology, 20139 Milan, Italy)

  • Noemi Rudini

    (IFOM Fondazione, FIRC Institute of Molecular Oncology, 20139 Milan, Italy)

  • Roberto Cuttano

    (IFOM Fondazione, FIRC Institute of Molecular Oncology, 20139 Milan, Italy)

  • Luca Bravi

    (IFOM Fondazione, FIRC Institute of Molecular Oncology, 20139 Milan, Italy)

  • Costanza Giampietro

    (IFOM Fondazione, FIRC Institute of Molecular Oncology, 20139 Milan, Italy)

  • Monica Corada

    (IFOM Fondazione, FIRC Institute of Molecular Oncology, 20139 Milan, Italy)

  • Luca Ferrarini

    (IFOM Fondazione, FIRC Institute of Molecular Oncology, 20139 Milan, Italy)

  • Fabrizio Orsenigo

    (IFOM Fondazione, FIRC Institute of Molecular Oncology, 20139 Milan, Italy)

  • Eleanna Papa

    (IFOM Fondazione, FIRC Institute of Molecular Oncology, 20139 Milan, Italy)

  • Gwenola Boulday

    (INSERM, UMR-S740, F-75010 Paris, France
    Université Paris Diderot, Sorbonne Paris Cité, Génétique des Maladies Vasculaires UMR-S740, F-75010 Paris, France)

  • Elisabeth Tournier-Lasserve

    (INSERM, UMR-S740, F-75010 Paris, France
    Université Paris Diderot, Sorbonne Paris Cité, Génétique des Maladies Vasculaires UMR-S740, F-75010 Paris, France
    AP-HP, Groupe Hospitalier Saint-Louis Lariboisiere-Fernand-Widal, F-75010 Paris, France)

  • Françoise Chapon

    (Centre Hospitalier Universitaire, Avenue Côte de Nacre, 14032 Caen, France)

  • Cristina Richichi

    (IEO – European Institute of Oncology, 20139 Milan, Italy)

  • Saverio Francesco Retta

    (University of Turin, 10043 Orbassano, Turin, Italy)

  • Maria Grazia Lampugnani

    (IFOM Fondazione, FIRC Institute of Molecular Oncology, 20139 Milan, Italy
    Mario Negri Institute of Pharmacological Research, 20156 Milan, Italy)

  • Elisabetta Dejana

    (IFOM Fondazione, FIRC Institute of Molecular Oncology, 20139 Milan, Italy
    University of Milan, 20133 Milan, Italy)

Abstract

Cerebral cavernous malformations associated with loss of function of Ccm1 are shown to be formed by endothelial cells undergoing endothelial-to-mesenchymal transition (EndMT) induced by TGF-β and BMP signalling; inhibition of TGF-β and BMP signalling prevents EndMT and the appearance of CCM lesions.

Suggested Citation

  • Luigi Maddaluno & Noemi Rudini & Roberto Cuttano & Luca Bravi & Costanza Giampietro & Monica Corada & Luca Ferrarini & Fabrizio Orsenigo & Eleanna Papa & Gwenola Boulday & Elisabeth Tournier-Lasserve , 2013. "EndMT contributes to the onset and progression of cerebral cavernous malformations," Nature, Nature, vol. 498(7455), pages 492-496, June.
    • Handle: RePEc:nat:nature:v:498:y:2013:i:7455:d:10.1038_nature12207
    DOI: 10.1038/nature12207
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    Cited by:

    1. Haifeng Zhang & Busu Li & Qunhua Huang & Francesc López-Giráldez & Yoshiaki Tanaka & Qun Lin & Sameet Mehta & Guilin Wang & Morven Graham & Xinran Liu & In-Hyun Park & Anne Eichmann & Wang Min & Jenny, 2022. "Mitochondrial dysfunction induces ALK5-SMAD2-mediated hypovascularization and arteriovenous malformations in mouse retinas," Nature Communications, Nature, vol. 13(1), pages 1-21, December.

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