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<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Endothelial ERK signaling controls lymphatic fate specification</title><author><name sortKey="Deng, Yong" sort="Deng, Yong" uniqKey="Deng Y" first="Yong" last="Deng">Yong Deng</name><affiliation><nlm:aff id="JCI63034">Yale Cardiovascular Research Center, Section of Cardiovascular Medicine, Department of Internal Medicine, Yale University School of Medicine, New Haven, Connecticut, USA.</nlm:aff></affiliation></author><author><name sortKey="Atri, Deepak" sort="Atri, Deepak" uniqKey="Atri D" first="Deepak" last="Atri">Deepak Atri</name><affiliation><nlm:aff id="JCI63034">Yale Cardiovascular Research Center, Section of Cardiovascular Medicine, Department of Internal Medicine, Yale University School of Medicine, New Haven, Connecticut, USA.</nlm:aff></affiliation></author><author><name sortKey="Eichmann, Anne" sort="Eichmann, Anne" uniqKey="Eichmann A" first="Anne" last="Eichmann">Anne Eichmann</name><affiliation><nlm:aff id="JCI63034">Yale Cardiovascular Research Center, Section of Cardiovascular Medicine, Department of Internal Medicine, Yale University School of Medicine, New Haven, Connecticut, USA.</nlm:aff></affiliation><affiliation><nlm:aff id="JCI63034">Center for Interdisciplinary Research in Biology (CIRB), Collège de France, Paris, France.</nlm:aff></affiliation></author><author><name sortKey="Simons, Michael" sort="Simons, Michael" uniqKey="Simons M" first="Michael" last="Simons">Michael Simons</name><affiliation><nlm:aff id="JCI63034">Yale Cardiovascular Research Center, Section of Cardiovascular Medicine, Department of Internal Medicine, Yale University School of Medicine, New Haven, Connecticut, USA.</nlm:aff></affiliation><affiliation><nlm:aff id="JCI63034">Department of Cell Biology, Yale University School of Medicine, New Haven, Connecticut, USA.</nlm:aff></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">PMC</idno><idno type="pmid">23391722</idno><idno type="pmc">3582116</idno><idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3582116</idno><idno type="RBID">PMC:3582116</idno><idno type="doi">10.1172/JCI63034</idno><date when="2013">2013</date><idno type="wicri:Area/Pmc/Corpus">002F20</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Corpus" wicri:corpus="PMC">002F20</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">Endothelial ERK signaling controls lymphatic fate specification</title><author><name sortKey="Deng, Yong" sort="Deng, Yong" uniqKey="Deng Y" first="Yong" last="Deng">Yong Deng</name><affiliation><nlm:aff id="JCI63034">Yale Cardiovascular Research Center, Section of Cardiovascular Medicine, Department of Internal Medicine, Yale University School of Medicine, New Haven, Connecticut, USA.</nlm:aff></affiliation></author><author><name sortKey="Atri, Deepak" sort="Atri, Deepak" uniqKey="Atri D" first="Deepak" last="Atri">Deepak Atri</name><affiliation><nlm:aff id="JCI63034">Yale Cardiovascular Research Center, Section of Cardiovascular Medicine, Department of Internal Medicine, Yale University School of Medicine, New Haven, Connecticut, USA.</nlm:aff></affiliation></author><author><name sortKey="Eichmann, Anne" sort="Eichmann, Anne" uniqKey="Eichmann A" first="Anne" last="Eichmann">Anne Eichmann</name><affiliation><nlm:aff id="JCI63034">Yale Cardiovascular Research Center, Section of Cardiovascular Medicine, Department of Internal Medicine, Yale University School of Medicine, New Haven, Connecticut, USA.</nlm:aff></affiliation><affiliation><nlm:aff id="JCI63034">Center for Interdisciplinary Research in Biology (CIRB), Collège de France, Paris, France.</nlm:aff></affiliation></author><author><name sortKey="Simons, Michael" sort="Simons, Michael" uniqKey="Simons M" first="Michael" last="Simons">Michael Simons</name><affiliation><nlm:aff id="JCI63034">Yale Cardiovascular Research Center, Section of Cardiovascular Medicine, Department of Internal Medicine, Yale University School of Medicine, New Haven, Connecticut, USA.</nlm:aff></affiliation><affiliation><nlm:aff id="JCI63034">Department of Cell Biology, Yale University School of Medicine, New Haven, Connecticut, USA.</nlm:aff></affiliation></author></analytic><series><title level="j">The Journal of Clinical Investigation</title><idno type="ISSN">0021-9738</idno><idno type="eISSN">1558-8238</idno><imprint><date when="2013">2013</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en"><p>Lymphatic vessels are thought to arise from PROX1-positive endothelial cells (ECs) in the cardinal vein in response to induction of SOX18 expression; however, the molecular event responsible for increased SOX18 expression has not been established. We generated mice with endothelial-specific, inducible expression of an <italic>RAF1</italic> gene with a gain-of-function mutation (<italic>RAF1<sup>S259A</sup></italic>) that is associated with Noonan syndrome. Expression of mutant <italic>RAF1<sup>S259A</sup></italic> in ECs activated ERK and induced SOX18 and PROX1 expression, leading to increased commitment of venous ECs to the lymphatic fate. Excessive production of lymphatic ECs resulted in lymphangiectasia that was highly reminiscent of abnormal lymphatics seen in Noonan syndrome and similar “RASopathies.” Inhibition of ERK signaling during development abrogated the lymphatic differentiation program and rescued the lymphatic phenotypes induced by expression of <italic>RAF1<sup>S259A</sup></italic>. These data suggest that ERK activation plays a key role in lymphatic EC fate specification and that excessive ERK activation is the basis of lymphatic abnormalities seen in Noonan syndrome and related diseases.
</p></div></front></TEI><pmc article-type="research-article"><pmc-comment>The publisher of this article does not allow downloading of the full text in XML form.</pmc-comment>
<front><journal-meta><journal-id journal-id-type="nlm-ta">J Clin Invest</journal-id><journal-id journal-id-type="iso-abbrev">J. Clin. Invest</journal-id><journal-id journal-id-type="publisher-id">J CLIN INVEST</journal-id><journal-title-group><journal-title>The Journal of Clinical Investigation</journal-title></journal-title-group><issn pub-type="ppub">0021-9738</issn><issn pub-type="epub">1558-8238</issn><publisher><publisher-name>American Society for Clinical Investigation</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="pmid">23391722</article-id><article-id pub-id-type="pmc">3582116</article-id><article-id pub-id-type="publisher-id">63034</article-id><article-id pub-id-type="doi">10.1172/JCI63034</article-id><article-categories><subj-group subj-group-type="heading"><subject>Research Article</subject></subj-group></article-categories><title-group><article-title>Endothelial ERK signaling controls lymphatic fate specification</article-title></title-group><contrib-group><contrib contrib-type="author"><name><surname>Deng</surname><given-names>Yong</given-names></name><xref ref-type="aff" rid="JCI63034">1</xref></contrib><contrib contrib-type="author"><name><surname>Atri</surname><given-names>Deepak</given-names></name><xref ref-type="aff" rid="JCI63034">1</xref></contrib><contrib contrib-type="author"><name><surname>Eichmann</surname><given-names>Anne</given-names></name><xref ref-type="aff" rid="JCI63034">1</xref><xref ref-type="aff" rid="JCI63034">2</xref></contrib><contrib contrib-type="author"><name><surname>Simons</surname><given-names>Michael</given-names></name><xref ref-type="aff" rid="JCI63034">1</xref><xref ref-type="aff" rid="JCI63034">3</xref></contrib></contrib-group><aff id="JCI63034"><label>1</label>Yale Cardiovascular Research Center, Section of Cardiovascular Medicine, Department of Internal Medicine, Yale University School of Medicine, New Haven, Connecticut, USA.<label>2</label>Center for Interdisciplinary Research in Biology (CIRB), Collège de France, Paris, France.<label>3</label>Department of Cell Biology, Yale University School of Medicine, New Haven, Connecticut, USA.</aff><author-notes><corresp>Address correspondence to: Michael Simons, Section of Cardiovascular Medicine, Yale University School of Medicine, 333 Cedar St., New Haven, Connecticut 06510, USA. Phone: 203.785.7000; Fax: 203.785.5144; E-mail:
<email>michael.simons@yale.edu</email>.
</corresp></author-notes><pub-date pub-type="epub"><day>8</day><month>2</month><year>2013</year></pub-date><pub-date pub-type="ppub"><day>1</day><month>3</month><year>2013</year></pub-date><pub-date pub-type="pmc-release"><day>8</day><month>2</month><year>2013</year></pub-date><pmc-comment> PMC Release delay is 0 months and 0 days and was based on the
<volume>123</volume><issue>3</issue><fpage>1202</fpage><lpage>1215</lpage><history><date date-type="received"><day>24</day><month>8</month><year>2012</year></date><date date-type="accepted"><day>13</day><month>12</month><year>2012</year></date></history><permissions><copyright-statement>Copyright © 2013, American Society for Clinical Investigation</copyright-statement><copyright-year>2013</copyright-year></permissions><abstract><p>Lymphatic vessels are thought to arise from PROX1-positive endothelial cells (ECs) in the cardinal vein in response to induction of SOX18 expression; however, the molecular event responsible for increased SOX18 expression has not been established. We generated mice with endothelial-specific, inducible expression of an <italic>RAF1</italic> gene with a gain-of-function mutation (<italic>RAF1<sup>S259A</sup></italic>) that is associated with Noonan syndrome. Expression of mutant <italic>RAF1<sup>S259A</sup></italic> in ECs activated ERK and induced SOX18 and PROX1 expression, leading to increased commitment of venous ECs to the lymphatic fate. Excessive production of lymphatic ECs resulted in lymphangiectasia that was highly reminiscent of abnormal lymphatics seen in Noonan syndrome and similar “RASopathies.” Inhibition of ERK signaling during development abrogated the lymphatic differentiation program and rescued the lymphatic phenotypes induced by expression of <italic>RAF1<sup>S259A</sup></italic>. These data suggest that ERK activation plays a key role in lymphatic EC fate specification and that excessive ERK activation is the basis of lymphatic abnormalities seen in Noonan syndrome and related diseases.
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