Leiden University Scholarly Publications

Persistent URL of this record https://hdl.handle.net/1887/108838

In Collections

This item can be found in the following collections:

Bokenkamp, R.; DeRuiter, M.C.; Munsteren, C. van; Gittenberger-de Groot, A.C. (2010)

Insights into the Pathogenesis and Genetic Background of Patency of the Ductus Arteriosus

Article / Letter to editor
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...Show moreThe 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. Copyright (C) 2009 S. Karger AG, BaselShow less
Ductus arteriosus Ductus arteriosus regulation, animal models Ductus arteriosus remodeling, animal models Persistent ductus arteriosus syndromic/non-syndromic Vascular smooth muscle cells congenital heart-disease endothelin-receptor blockade oxygen-induced constriction permanent anatomic closure nitric-oxide synthase smooth-muscle-cells cause char-syndrome in-vivo neointimal formation k+ channels
All authors
Bokenkamp, R.; DeRuiter, M.C.; Munsteren, C. van; Gittenberger-de Groot, A.C.
Date
2010-12-31
Journal
Neonatology
Volume
98
Issue
1
Pages
6 - 17