title: Heart valve structure and function in development and disease.
abstract: The mature heart valves are made up of highly organized extracellular matrix (ECM) and valve interstitial cells (VICs) surrounded by an endothelial cell layer. The ECM of the valves is stratified into elastin-, proteoglycan-, and collagen-rich layers that confer distinct biomechanical properties to the leaflets and supporting structures. Signaling pathways have critical functions in primary valvulogenesis as well as the maintenance of valve structure and function over time. Animal models provide powerful tools to study valve development and disease processes. Valve disease is a significant public health problem, and increasing evidence implicates aberrant developmental mechanisms underlying pathogenesis. Further studies are necessary to determine regulatory pathway interactions underlying valve pathogenesis in order to generate new avenues for novel therapeutics.
authors.affiliation: Division of Cardiology, The Heart Institute, Cincinnati Children's Hospital Medical Center, Ohio 45229, USA.
authors.forename: Robert B
authors.initials: RB
authors.lastname: Hinton
authors.forename: Katherine E
authors.initials: KE
authors.lastname: Yutzey
mesh_terms: D000818:Animals; D003094:Collagen / Q000502:physiology; D042783:Endothelial Cells / Q000502:physiology; D005109:Extracellular Matrix / Q000502:physiology; D018507:Gene Expression Regulation, Developmental / Q000502:physiology; D006331:Heart Diseases / Q000235:genetics / Q000503:physiopathology*; D006351:Heart Valves / Q000502:physiology / Q000503:physiopathology / Q000648:ultrastructure; D006801:Humans; D051379:Mice; D015398:Signal Transduction / Q000502:physiology
chemical_list: D003094:Collagen
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