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dc.contributor.author Zhou, Zinan
dc.contributor.author Rawnsley, David R
dc.contributor.author Goddard, Lauren M
dc.contributor.author Pan, Wei
dc.contributor.author Cao, Xin-Jung
dc.contributor.author Jakus, Zoltán
dc.contributor.author Hui, Zheng
dc.contributor.author Jisheng, Yang
dc.contributor.author Simon, Arthur
dc.contributor.author Kevin, J Whitehead
dc.contributor.author Dean, Li
dc.contributor.author Bin, Zhou
dc.contributor.author Benjamin, A Garcia
dc.contributor.author Xiangjian, Zheng
dc.contributor.author Mark, L Kahn
dc.date.accessioned 2017-01-12T12:36:08Z
dc.date.available 2017-01-12T12:36:08Z
dc.date.issued 2015
dc.identifier.citation pagination=168-180; journalVolume=32; journalIssueNumber=2; journalTitle=DEVELOPMENTAL CELL;
dc.identifier.uri http://repo.lib.semmelweis.hu//handle/123456789/1319
dc.identifier.uri doi:10.1016/j.devcel.2014.12.009
dc.description.abstract The cerebral cavernous malformation (CCM) pathway is required in endothelial cells for normal cardiovascular development and to prevent postnatal vascular malformations, but its molecular effectors are not well defined. Here we show that loss of CCM signaling in endocardial cells results in mid-gestation heart failure associated with premature degradation of cardiac jelly. CCM deficiency dramatically alters endocardial and endothelial gene expression, including increased expression of the Klf2 and Klf4 transcription factors and the Adamts4 and Adamts5 proteases that degrade cardiac jelly. These changes in gene expression result from increased activity of MEKK3, a mitogen-activated protein kinase that binds CCM2 in endothelial cells. MEKK3 is both necessary and sufficient for expression of these genes, and partial loss of MEKK3 rescues cardiac defects in CCM-deficient embryos. These findings reveal a molecular mechanism by which CCM signaling controls endothelial gene expression during cardiovascular development that may also underlie CCM formation.
dc.relation.ispartof urn:issn:1534-5807
dc.title The Cerebral Cavernous Malformation Pathway Controls Cardiac Development via Regulation of Endocardial MEKK3 Signaling and KLF Expression
dc.type Journal Article
dc.date.updated 2015-02-04T10:10:23Z
dc.language.rfc3066 en
dc.identifier.mtmt 2828119
dc.identifier.pubmed 25625206


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