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Endothelial cell clonal expansion in the development of cerebral cavernous malformations
FIRC Inst Mol Oncol Fdn, Vasc Biol Unit, I-20139 Milan, Italy.
FIRC Inst Mol Oncol Fdn, Vasc Biol Unit, I-20139 Milan, Italy.
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Vascular Biology.
FIRC Inst Mol Oncol Fdn, Vasc Biol Unit, I-20139 Milan, Italy.
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2019 (English)In: Nature Communications, ISSN 2041-1723, E-ISSN 2041-1723, Vol. 10, article id 2761Article in journal (Refereed) Published
Abstract [en]

Cerebral cavernous malformation (CCM) is a neurovascular familial or sporadic disease that is characterised by capillary-venous cavernomas, and is due to loss-of-function mutations to any one of three CCM genes. Familial CCM follows a two-hit mechanism similar to that of tumour suppressor genes, while in sporadic cavernomas only a small fraction of endothelial cells shows mutated CCM genes. We reported that in mouse models and in human patients, endothelial cells lining the lesions have different features from the surrounding endothelium, as they express mesenchymal/stem-cell markers. Here we show that cavernomas originate from clonal expansion of few Ccm3-null endothelial cells that express mesenchymal/stem-cell markers. These cells then attract surrounding wild-type endothelial cells, inducing them to express mesenchymal/stem-cell markers and to contribute to cavernoma growth. These characteristics of Ccm3-null cells are reminiscent of the tumour-initiating cells that are responsible for tumour growth. Our data support the concept that CCM has benign tumour characteristics.

Place, publisher, year, edition, pages
NATURE PUBLISHING GROUP , 2019. Vol. 10, article id 2761
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Cell and Molecular Biology
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URN: urn:nbn:se:uu:diva-390826DOI: 10.1038/s41467-019-10707-xISI: 000472598000002PubMedID: 31235698OAI: oai:DiVA.org:uu-390826DiVA, id: diva2:1342897
Funder
Swedish Research Council, 2013-9279Knut and Alice Wallenberg FoundationEU, European Research Council, 742922Available from: 2019-08-14 Created: 2019-08-14 Last updated: 2019-08-14Bibliographically approved

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