ccm1 cell autonomously regulates endothelial cellular morphogenesis and vascular tubulogenesis in zebrafish

Hogan, Benjamin M., Bussmann, Jeroen, Wolburg, Hartwig and Schulte-Merker, Stefan (2008) ccm1 cell autonomously regulates endothelial cellular morphogenesis and vascular tubulogenesis in zebrafish. Human Molecular Genetics, 17 16: 2424-2432. doi:10.1093/hmg/ddn142


Author Hogan, Benjamin M.
Bussmann, Jeroen
Wolburg, Hartwig
Schulte-Merker, Stefan
Title ccm1 cell autonomously regulates endothelial cellular morphogenesis and vascular tubulogenesis in zebrafish
Journal name Human Molecular Genetics   Check publisher's open access policy
ISSN 0964-6906
1460-2083
Publication date 2008
Year available 2008
Sub-type Article (original research)
DOI 10.1093/hmg/ddn142
Open Access Status
Volume 17
Issue 16
Start page 2424
End page 2432
Total pages 9
Place of publication Oxford, United Kingdom
Publisher Oxford University Press
Collection year 2008
Language eng
Subject 1311 Genetics
Abstract Cerebral cavernous malformations (CCMs) are a prevalent class of vascular anomalies characterized by thin-walled clusters of malformed blood vessels in the brain. Heritable forms are caused by mutations in CCM1, CCM2 and CCM3, but despite the importance of these factors in vascular biology, an understanding of their molecular and cellular functions remains elusive. Here we describe the characterization of a zebrafish embryonic model of CCM. Loss of ccm1 in zebrafish embryos leads to severe and progressive dilation of major vessels, despite normal endothelial cell fate and number. Vascular dilation in ccm1 mutants is accompanied by progressive spreading of endothelial cells and thinning of vessel walls despite ultrastructurally normal cell-cell contacts. Zebrafish ccm2 mutants display comparable vascular defects. Finally, we show that ccm1 function is cell autonomous, suggesting that it is endothelial cellular morphogenesis that is regulated by CCM proteins during development and pathogenesis.
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status Non-UQ

Document type: Journal Article
Sub-type: Article (original research)
Collection: Institute for Molecular Bioscience - Publications
 
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