title: Endocardial Notch Signaling in Cardiac Development and Disease.
abstract: The Notch signaling pathway is an ancient and highly conserved signaling pathway that controls cell fate specification and tissue patterning in the embryo and in the adult. Region-specific endocardial Notch activity regulates heart morphogenesis through the interaction with multiple myocardial-, epicardial-, and neural crest-derived signals. Mutations in NOTCH signaling elements cause congenital heart disease in humans and mice, demonstrating its essential role in cardiac development. Studies in model systems have provided mechanistic understanding of Notch function in cardiac development, congenital heart disease, and heart regeneration. Notch patterns the embryonic endocardium into prospective territories for valve and chamber formation, and later regulates the signaling processes leading to outflow tract and valve morphogenesis and ventricular trabeculae compaction. Alterations in NOTCH signaling in the endocardium result in congenital structural malformations that can lead to disease in the neonate and adult heart.
authors.affiliation: From the Intercellular Signaling in Cardiovascular Development and Disease Laboratory, Centro Nacional de Investigaciones Cardiovascular (CNIC), Melchor Fernández Almagro, Madrid, Spain (G.L., G.D'A., D.M., J.L.d.l.P.); and Department of Tissue Morphogenesis, Max Planck Institute for Molecular Biomedicine, Münster, Germany (G.L.).
authors.forename: Guillermo
authors.initials: G
authors.lastname: Luxán
authors.affiliation: From the Intercellular Signaling in Cardiovascular Development and Disease Laboratory, Centro Nacional de Investigaciones Cardiovascular (CNIC), Melchor Fernández Almagro, Madrid, Spain (G.L., G.D'A., D.M., J.L.d.l.P.); and Department of Tissue Morphogenesis, Max Planck Institute for Molecular Biomedicine, Münster, Germany (G.L.).
authors.forename: Gaetano
authors.initials: G
authors.lastname: D'Amato
authors.affiliation: From the Intercellular Signaling in Cardiovascular Development and Disease Laboratory, Centro Nacional de Investigaciones Cardiovascular (CNIC), Melchor Fernández Almagro, Madrid, Spain (G.L., G.D'A., D.M., J.L.d.l.P.); and Department of Tissue Morphogenesis, Max Planck Institute for Molecular Biomedicine, Münster, Germany (G.L.).
authors.forename: Donal
authors.initials: D
authors.lastname: MacGrogan
authors.affiliation: From the Intercellular Signaling in Cardiovascular Development and Disease Laboratory, Centro Nacional de Investigaciones Cardiovascular (CNIC), Melchor Fernández Almagro, Madrid, Spain (G.L., G.D'A., D.M., J.L.d.l.P.); and Department of Tissue Morphogenesis, Max Planck Institute for Molecular Biomedicine, Münster, Germany (G.L.). jlpompa@cnic.es.
authors.forename: José Luis
authors.initials: JL
authors.lastname: de la Pompa
mesh_terms: D000818:Animals; D004699:Endocardium / Q000254:growth & development* / Q000378:metabolism* / Q000473:pathology; D006330:Heart Defects, Congenital / Q000378:metabolism / Q000473:pathology; D006331:Heart Diseases / Q000378:metabolism* / Q000473:pathology; D006801:Humans; D051880:Receptors, Notch / Q000378:metabolism*; D015398:Signal Transduction / Q000502:physiology*
chemical_list: D051880:Receptors, Notch
keywords: Notch signaling; cardiomyopathies; endocardium; heart valves; heart ventricles