EndMT contributes to the onset and progression of cerebral cavernous malformations

Luigi Maddaluno (), Noemi Rudini, Roberto Cuttano, Luca Bravi, Costanza Giampietro, Monica Corada, Luca Ferrarini, Fabrizio Orsenigo, Eleanna Papa, Gwenola Boulday, Elisabeth Tournier-Lasserve, Françoise Chapon, Cristina Richichi, Saverio Francesco Retta, Maria Grazia Lampugnani and Elisabetta Dejana ()
Additional contact information
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
Elisabeth Tournier-Lasserve: INSERM, UMR-S740, 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
Elisabetta Dejana: IFOM Fondazione, FIRC Institute of Molecular Oncology, 20139 Milan, Italy

Nature, 2013, vol. 498, issue 7455, 492-496

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.

Date: 2013
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DOI: 10.1038/nature12207

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