| Insights into the pathogenesis and genetic background of patency of the ductus arteriosus. | |
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MedLine Citation:
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PMID: 19955832 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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The unique differentiation program of the ductus arteriosus (DA) is essential for its specific task during fetal life and for the adapting circulation after birth. Phenotypic changes occur in the DA during the normal maturation and definitive closure. Morphological abnormalities of the vessel wall characterize the persistent DA (PDA) in older children. Here, we give an overview of the animal models of DA regulation and remodeling. Genetic research has identified the cause of syndromic forms of PDA, such as the TFAP2B mutations in Char syndrome. Genes that interfere with the remodeling of vascular smooth muscle cells (VSMCs) of the ductal media are affected in virtually all of these anomalies. Therefore, the pivotal regulatory role of VSMCs is emphasized. A better understanding of the genetic background of this developmental process may help develop new strategies to manipulate the DA in premature infants, neonates with duct-dependent anomalies, and patients with syndromic and non-syndromic PDA. |
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Authors:
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Regina Bökenkamp; Marco C DeRuiter; Conny van Munsteren; Adriana C Gittenberger-de Groot |
Publication Detail:
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Type: Journal Article; Review Date: 2009-12-02 |
Journal Detail:
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Title: Neonatology Volume: 98 ISSN: 1661-7819 ISO Abbreviation: Neonatology Publication Date: 2010 Jun |
Date Detail:
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Created Date: 2010-06-30 Completed Date: 2010-10-14 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 101286577 Medline TA: Neonatology Country: Switzerland |
Other Details:
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Languages: eng Pagination: 6-17 Citation Subset: IM |
Affiliation:
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Department of Pediatric Cardiology , Leiden University Medical Center, Leiden, The Netherlands. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Animals Disease Models, Animal Dogs Ductus Arteriosus, Patent / etiology*, genetics* Humans Infant, Newborn Infant, Premature / growth & development Mice Muscle, Smooth, Vascular / growth & development* Mutation Rabbits Rats Transcription Factor AP-2 / genetics |
| Chemical | |
Reg. No./Substance:
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0/TFAP2B protein, human; 0/Transcription Factor AP-2 |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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