{"@context":{"@vocab":"https://cir.nii.ac.jp/schema/1.0/","rdfs":"http://www.w3.org/2000/01/rdf-schema#","dc":"http://purl.org/dc/elements/1.1/","dcterms":"http://purl.org/dc/terms/","foaf":"http://xmlns.com/foaf/0.1/","prism":"http://prismstandard.org/namespaces/basic/2.0/","cinii":"http://ci.nii.ac.jp/ns/1.0/","datacite":"https://schema.datacite.org/meta/kernel-4/","ndl":"http://ndl.go.jp/dcndl/terms/","jpcoar":"https://github.com/JPCOAR/schema/blob/master/2.0/"},"@id":"https://cir.nii.ac.jp/crid/1361699995484883200.json","@type":"Article","productIdentifier":[{"identifier":{"@type":"DOI","@value":"10.1161/01.res.0000026056.81424.da"}},{"identifier":{"@type":"URI","@value":"https://www.ahajournals.org/doi/full/10.1161/01.RES.0000026056.81424.DA"}}],"dc:title":[{"@value":"Hyperplastic Conotruncal Endocardial Cushions and Transposition of Great Arteries in Perlecan-Null Mice"}],"description":[{"type":"abstract","notation":[{"@value":"<jats:p>Perlecan is a heparan-sulfate proteoglycan abundantly expressed in pericellular matrices and basement membranes during development. Inactivation of the perlecan gene in mice is lethal at two developmental stages: around E10 and around birth. We report a high incidence of malformations of the cardiac outflow tract in perlecan-deficient embryos. Complete transposition of great arteries was diagnosed in 11 out of 15 late embryos studied (73%). Three of these 11 embryos also showed malformations of semilunar valves. Mesenchymal cells in the outflow tract were abnormally abundant in mutant embryos by E9.5, when the endocardial-mesenchymal transformation starts in wild-type embryos. At E10.5, mutant embryos lacked well-defined spiral endocardial ridges, and the excess of mesenchymal cells obstructed sometimes the outflow tract lumen. Most of this anomalous mesenchyme expressed the smooth muscle cell-specific α-actin isoform, a marker of the neural crest in the outflow tract of the mouse. In wild-type embryos, perlecan is present in the basal surface of myocardium and endocardium, as well as surrounding presumptive neural crest cells. We suggest that the excess of mesenchyme at the earlier stages of conotruncal development precludes the formation of the spiral ridges and the rotation of the septation complex in order to achieve a concordant ventriculoarterial connection. The observed mesenchymal overpopulation might be due to an uncontrolled migration of neural crest cells, which would arrive prematurely to the heart. Thus, perlecan is involved in the control of the outflow tract mesenchymal population size, underscoring the importance of the extracellular matrix in cardiac morphogenesis.</jats:p>"}]}],"creator":[{"@id":"https://cir.nii.ac.jp/crid/1381699995484883201","@type":"Researcher","foaf:name":[{"@value":"Mercedes Costell"}],"jpcoar:affiliationName":[{"@value":"From the Department of Biochemistry and Molecular Biology (M.C.), University of Valencia, Valencia, Spain; the Department of Animal Biology (R.C., M.G.-I., R.M.-C.), University of Málaga, Málaga, Spain; and the Department of Experimental Pathology (E.G., R.F.), Lund University, Lund, Sweden."}]},{"@id":"https://cir.nii.ac.jp/crid/1381699995484883202","@type":"Researcher","foaf:name":[{"@value":"Rita Carmona"}],"jpcoar:affiliationName":[{"@value":"From the Department of Biochemistry and Molecular Biology (M.C.), University of Valencia, Valencia, Spain; the Department of Animal Biology (R.C., M.G.-I., R.M.-C.), University of Málaga, Málaga, Spain; and the Department of Experimental Pathology (E.G., R.F.), Lund University, Lund, Sweden."}]},{"@id":"https://cir.nii.ac.jp/crid/1381699995484883203","@type":"Researcher","foaf:name":[{"@value":"Erika Gustafsson"}],"jpcoar:affiliationName":[{"@value":"From the Department of Biochemistry and Molecular Biology (M.C.), University of Valencia, Valencia, Spain; the Department of Animal Biology (R.C., M.G.-I., R.M.-C.), University of Málaga, Málaga, Spain; and the Department of Experimental Pathology (E.G., R.F.), Lund University, Lund, Sweden."}]},{"@id":"https://cir.nii.ac.jp/crid/1381699995484883328","@type":"Researcher","foaf:name":[{"@value":"Mauricio González-Iriarte"}],"jpcoar:affiliationName":[{"@value":"From the Department of Biochemistry and Molecular Biology (M.C.), University of Valencia, Valencia, Spain; the Department of Animal Biology (R.C., M.G.-I., R.M.-C.), University of Málaga, Málaga, Spain; and the Department of Experimental Pathology (E.G., R.F.), Lund University, Lund, Sweden."}]},{"@id":"https://cir.nii.ac.jp/crid/1381699995484883200","@type":"Researcher","foaf:name":[{"@value":"Reinhard Fässler"}],"jpcoar:affiliationName":[{"@value":"From the Department of Biochemistry and Molecular Biology (M.C.), University of Valencia, Valencia, Spain; the Department of Animal Biology (R.C., M.G.-I., R.M.-C.), University of Málaga, Málaga, Spain; and the Department of Experimental Pathology (E.G., R.F.), Lund University, Lund, Sweden."}]},{"@id":"https://cir.nii.ac.jp/crid/1381699995484883204","@type":"Researcher","foaf:name":[{"@value":"Ramón Muñoz-Chápuli"}],"jpcoar:affiliationName":[{"@value":"From the Department of Biochemistry and Molecular Biology (M.C.), University of Valencia, Valencia, Spain; the Department of Animal Biology (R.C., M.G.-I., R.M.-C.), University of Málaga, Málaga, Spain; and the Department of Experimental Pathology (E.G., R.F.), Lund University, Lund, Sweden."}]}],"publication":{"publicationIdentifier":[{"@type":"PISSN","@value":"00097330"},{"@type":"EISSN","@value":"15244571"}],"prism:publicationName":[{"@value":"Circulation Research"}],"dc:publisher":[{"@value":"Ovid Technologies (Wolters Kluwer Health)"}],"prism:publicationDate":"2002-07-26","prism:volume":"91","prism:number":"2","prism:startingPage":"158","prism:endingPage":"164"},"reviewed":"false","url":[{"@id":"https://www.ahajournals.org/doi/full/10.1161/01.RES.0000026056.81424.DA"}],"createdAt":"2002-10-01","modifiedAt":"2024-05-12","relatedProduct":[{"@id":"https://cir.nii.ac.jp/crid/1360004236977873024","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"HB-EGF function in cardiac valve development requires interaction with heparan sulfate proteoglycans"}]},{"@id":"https://cir.nii.ac.jp/crid/1360294643719217664","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Increased Risk of Aortic Dissection with Perlecan Deficiency"}]},{"@id":"https://cir.nii.ac.jp/crid/1360580232188704000","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Impact of the heparan sulfate proteoglycan perlecan on human disease and health"}]},{"@id":"https://cir.nii.ac.jp/crid/1360848662662982272","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Perlecan deficiency causes endothelial dysfunction by reducing the expression of endothelial nitric oxide synthase"}]}],"dataSourceIdentifier":[{"@type":"CROSSREF","@value":"10.1161/01.res.0000026056.81424.da"},{"@type":"CROSSREF","@value":"10.3390/ijms23010315_references_DOI_FKO0H6ZzsH2jrr77EdN3gzuPK9Q"},{"@type":"CROSSREF","@value":"10.1242/dev.048926_references_DOI_FKO0H6ZzsH2jrr77EdN3gzuPK9Q"},{"@type":"CROSSREF","@value":"10.1152/ajpcell.00113.2022_references_DOI_VBJImTocKkCBDkkWY61ZRltthGr"},{"@type":"CROSSREF","@value":"10.14814/phy2.12272_references_DOI_FKO0H6ZzsH2jrr77EdN3gzuPK9Q"}]}