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Tbx3 Is Required for Outflow Tract Development
Conotruncal and ventricular septal congenital heart anomalies result from defects in formation and division of the embryonic outflow tract. Cardiac remodeling during outflow tract and ventricular septation converts the tubular embryonic heart into a parallel circulatory system with an independent le...
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Published in: | Circulation research 2008-09, Vol.103 (7), p.743-750 |
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description | Conotruncal and ventricular septal congenital heart anomalies result from defects in formation and division of the embryonic outflow tract. Cardiac remodeling during outflow tract and ventricular septation converts the tubular embryonic heart into a parallel circulatory system with an independent left ventricular outlet and right ventricular inlet. Tbx3 encodes a T-box–containing transcription factor expressed in the developing conduction system of the heart. Mutations in TBX3 cause ulnar–mammary syndrome. Here we show that mice lacking Tbx3 develop severe outflow tract defects, including connection of both the aorta and pulmonary trunk with the right ventricle, in addition to aortic arch artery anomalies and abnormal communication between the right atrium and left ventricle. Alignment defects are preceded by a delay in caudal displacement of the arterial pole of the heart during aortic arch artery formation. Embryonic anterior–posterior patterning and cardiac chamber development are unaffected in Tbx3 mutant embryos. However, the contribution of second heart field derived progenitor cells to the arterial pole of the heart is impaired. Tbx3 is expressed in pharyngeal epithelia and neural crest cells in the pharyngeal region, suggesting an indirect role in second heart field deployment. Loss of Tbx3 affects multiple signaling pathways regulating second heart field proliferation and outflow tract morphogenesis, including fibroblast growth factor signaling, leading to a failure of normal heart tube extension and consequent atrioventricular and ventriculoarterial alignment defects. |
doi_str_mv | 10.1161/CIRCRESAHA.108.172858 |
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Cardiac remodeling during outflow tract and ventricular septation converts the tubular embryonic heart into a parallel circulatory system with an independent left ventricular outlet and right ventricular inlet. Tbx3 encodes a T-box–containing transcription factor expressed in the developing conduction system of the heart. Mutations in TBX3 cause ulnar–mammary syndrome. Here we show that mice lacking Tbx3 develop severe outflow tract defects, including connection of both the aorta and pulmonary trunk with the right ventricle, in addition to aortic arch artery anomalies and abnormal communication between the right atrium and left ventricle. Alignment defects are preceded by a delay in caudal displacement of the arterial pole of the heart during aortic arch artery formation. Embryonic anterior–posterior patterning and cardiac chamber development are unaffected in Tbx3 mutant embryos. However, the contribution of second heart field derived progenitor cells to the arterial pole of the heart is impaired. Tbx3 is expressed in pharyngeal epithelia and neural crest cells in the pharyngeal region, suggesting an indirect role in second heart field deployment. Loss of Tbx3 affects multiple signaling pathways regulating second heart field proliferation and outflow tract morphogenesis, including fibroblast growth factor signaling, leading to a failure of normal heart tube extension and consequent atrioventricular and ventriculoarterial alignment defects.</description><identifier>ISSN: 0009-7330</identifier><identifier>EISSN: 1524-4571</identifier><identifier>DOI: 10.1161/CIRCRESAHA.108.172858</identifier><identifier>PMID: 18723448</identifier><identifier>CODEN: CIRUAL</identifier><language>eng</language><publisher>Hagerstown, MD: American Heart Association, Inc</publisher><subject>Animals ; Aorta - embryology ; Biological and medical sciences ; Cellular Biology ; Fundamental and applied biological sciences. Psychology ; Gene Expression Regulation, Developmental - physiology ; Heart - embryology ; Heart Defects, Congenital - genetics ; Life Sciences ; Mice ; Mice, Mutant Strains ; Organ Specificity - physiology ; Organogenesis - physiology ; Signal Transduction - physiology ; T-Box Domain Proteins - genetics ; T-Box Domain Proteins - metabolism ; Vertebrates: cardiovascular system</subject><ispartof>Circulation research, 2008-09, Vol.103 (7), p.743-750</ispartof><rights>2008 American Heart Association, Inc.</rights><rights>2008 INIST-CNRS</rights><rights>Distributed under a Creative Commons Attribution 4.0 International License</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c6211-a0aaf699d22d87775800fa9d76769f090a7e0ba2193c916f320268d39713a14e3</citedby><cites>FETCH-LOGICAL-c6211-a0aaf699d22d87775800fa9d76769f090a7e0ba2193c916f320268d39713a14e3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>230,315,786,790,891,27957,27958</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=20702659$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/18723448$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink><backlink>$$Uhttps://hal.science/hal-00409363$$DView record in HAL$$Hfree_for_read</backlink></links><search><creatorcontrib>Mesbah, Karim</creatorcontrib><creatorcontrib>Harrelson, Zachary</creatorcontrib><creatorcontrib>Théveniau-Ruissy, Magali</creatorcontrib><creatorcontrib>Papaioannou, Virginia E</creatorcontrib><creatorcontrib>Kelly, Robert G</creatorcontrib><title>Tbx3 Is Required for Outflow Tract Development</title><title>Circulation research</title><addtitle>Circ Res</addtitle><description>Conotruncal and ventricular septal congenital heart anomalies result from defects in formation and division of the embryonic outflow tract. Cardiac remodeling during outflow tract and ventricular septation converts the tubular embryonic heart into a parallel circulatory system with an independent left ventricular outlet and right ventricular inlet. Tbx3 encodes a T-box–containing transcription factor expressed in the developing conduction system of the heart. Mutations in TBX3 cause ulnar–mammary syndrome. Here we show that mice lacking Tbx3 develop severe outflow tract defects, including connection of both the aorta and pulmonary trunk with the right ventricle, in addition to aortic arch artery anomalies and abnormal communication between the right atrium and left ventricle. Alignment defects are preceded by a delay in caudal displacement of the arterial pole of the heart during aortic arch artery formation. Embryonic anterior–posterior patterning and cardiac chamber development are unaffected in Tbx3 mutant embryos. However, the contribution of second heart field derived progenitor cells to the arterial pole of the heart is impaired. Tbx3 is expressed in pharyngeal epithelia and neural crest cells in the pharyngeal region, suggesting an indirect role in second heart field deployment. Loss of Tbx3 affects multiple signaling pathways regulating second heart field proliferation and outflow tract morphogenesis, including fibroblast growth factor signaling, leading to a failure of normal heart tube extension and consequent atrioventricular and ventriculoarterial alignment defects.</description><subject>Animals</subject><subject>Aorta - embryology</subject><subject>Biological and medical sciences</subject><subject>Cellular Biology</subject><subject>Fundamental and applied biological sciences. Psychology</subject><subject>Gene Expression Regulation, Developmental - physiology</subject><subject>Heart - embryology</subject><subject>Heart Defects, Congenital - genetics</subject><subject>Life Sciences</subject><subject>Mice</subject><subject>Mice, Mutant Strains</subject><subject>Organ Specificity - physiology</subject><subject>Organogenesis - physiology</subject><subject>Signal Transduction - physiology</subject><subject>T-Box Domain Proteins - genetics</subject><subject>T-Box Domain Proteins - metabolism</subject><subject>Vertebrates: cardiovascular system</subject><issn>0009-7330</issn><issn>1524-4571</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2008</creationdate><recordtype>article</recordtype><recordid>eNpdkV-LEzEUxYMobl39CMq8KPgw9d4kkz8vQqmrLRQWan0O6UxiR9NON5lp9dubZcqu-hQ4-Z1zufcQ8hphiijww3y5nq9vvs4WsymCmqKkqlJPyAQrykteSXxKJgCgS8kYXJEXKf0AQM6ofk6uUEnKOFcTMt1sf7FimYq1uxva6JrCd7G4HXofunOxibbui0_u5EJ33LtD_5I88zYk9-ryXpNvn28280W5uv2ynM9WZS0oYmnBWi-0bihtlJSyUgDe6kYKKbQHDVY62FqKmtUahWcUqFAN0xKZRe7YNfk45h6H7d41dR4dbTDH2O5t_G0625p_fw7tznzvToYBCFQ8B7wfA3b_2RazlbnXADhoJtgJM_vuMix2d4NLvdm3qXYh2IPrhmSEFsAEpxmsRrCOXUrR-YdkBHPfinlsJUvKjK1k35u_t3l0XWrIwNsLYFNtg4_2ULfpgaMg830qnTk-cucu9C6mn2E4u2h2zoZ-l1cCYIC0pAAKNBVQZgWR_QGfZqMk</recordid><startdate>20080926</startdate><enddate>20080926</enddate><creator>Mesbah, Karim</creator><creator>Harrelson, Zachary</creator><creator>Théveniau-Ruissy, Magali</creator><creator>Papaioannou, Virginia E</creator><creator>Kelly, Robert G</creator><general>American Heart Association, Inc</general><general>Lippincott</general><general>American Heart Association</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><scope>1XC</scope><scope>5PM</scope></search><sort><creationdate>20080926</creationdate><title>Tbx3 Is Required for Outflow Tract Development</title><author>Mesbah, Karim ; Harrelson, Zachary ; Théveniau-Ruissy, Magali ; Papaioannou, Virginia E ; Kelly, Robert G</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c6211-a0aaf699d22d87775800fa9d76769f090a7e0ba2193c916f320268d39713a14e3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2008</creationdate><topic>Animals</topic><topic>Aorta - embryology</topic><topic>Biological and medical sciences</topic><topic>Cellular Biology</topic><topic>Fundamental and applied biological sciences. Psychology</topic><topic>Gene Expression Regulation, Developmental - physiology</topic><topic>Heart - embryology</topic><topic>Heart Defects, Congenital - genetics</topic><topic>Life Sciences</topic><topic>Mice</topic><topic>Mice, Mutant Strains</topic><topic>Organ Specificity - physiology</topic><topic>Organogenesis - physiology</topic><topic>Signal Transduction - physiology</topic><topic>T-Box Domain Proteins - genetics</topic><topic>T-Box Domain Proteins - metabolism</topic><topic>Vertebrates: cardiovascular system</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Mesbah, Karim</creatorcontrib><creatorcontrib>Harrelson, Zachary</creatorcontrib><creatorcontrib>Théveniau-Ruissy, Magali</creatorcontrib><creatorcontrib>Papaioannou, Virginia E</creatorcontrib><creatorcontrib>Kelly, Robert G</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><collection>Hyper Article en Ligne (HAL)</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>Circulation research</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Mesbah, Karim</au><au>Harrelson, Zachary</au><au>Théveniau-Ruissy, Magali</au><au>Papaioannou, Virginia E</au><au>Kelly, Robert G</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Tbx3 Is Required for Outflow Tract Development</atitle><jtitle>Circulation research</jtitle><addtitle>Circ Res</addtitle><date>2008-09-26</date><risdate>2008</risdate><volume>103</volume><issue>7</issue><spage>743</spage><epage>750</epage><pages>743-750</pages><issn>0009-7330</issn><eissn>1524-4571</eissn><coden>CIRUAL</coden><notes>ObjectType-Article-1</notes><notes>SourceType-Scholarly Journals-1</notes><notes>ObjectType-Feature-2</notes><notes>content type line 23</notes><notes>Current address: Skaggs School of Pharmacy, University of California, San Diego, 9500 Gilman Drive, La Jolla, California, USA</notes><abstract>Conotruncal and ventricular septal congenital heart anomalies result from defects in formation and division of the embryonic outflow tract. Cardiac remodeling during outflow tract and ventricular septation converts the tubular embryonic heart into a parallel circulatory system with an independent left ventricular outlet and right ventricular inlet. Tbx3 encodes a T-box–containing transcription factor expressed in the developing conduction system of the heart. Mutations in TBX3 cause ulnar–mammary syndrome. Here we show that mice lacking Tbx3 develop severe outflow tract defects, including connection of both the aorta and pulmonary trunk with the right ventricle, in addition to aortic arch artery anomalies and abnormal communication between the right atrium and left ventricle. Alignment defects are preceded by a delay in caudal displacement of the arterial pole of the heart during aortic arch artery formation. Embryonic anterior–posterior patterning and cardiac chamber development are unaffected in Tbx3 mutant embryos. However, the contribution of second heart field derived progenitor cells to the arterial pole of the heart is impaired. Tbx3 is expressed in pharyngeal epithelia and neural crest cells in the pharyngeal region, suggesting an indirect role in second heart field deployment. Loss of Tbx3 affects multiple signaling pathways regulating second heart field proliferation and outflow tract morphogenesis, including fibroblast growth factor signaling, leading to a failure of normal heart tube extension and consequent atrioventricular and ventriculoarterial alignment defects.</abstract><cop>Hagerstown, MD</cop><pub>American Heart Association, Inc</pub><pmid>18723448</pmid><doi>10.1161/CIRCRESAHA.108.172858</doi><tpages>8</tpages><oa>free_for_read</oa></addata></record> |
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subjects | Animals Aorta - embryology Biological and medical sciences Cellular Biology Fundamental and applied biological sciences. Psychology Gene Expression Regulation, Developmental - physiology Heart - embryology Heart Defects, Congenital - genetics Life Sciences Mice Mice, Mutant Strains Organ Specificity - physiology Organogenesis - physiology Signal Transduction - physiology T-Box Domain Proteins - genetics T-Box Domain Proteins - metabolism Vertebrates: cardiovascular system |
title | Tbx3 Is Required for Outflow Tract Development |
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