{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,30]],"date-time":"2026-07-30T14:42:58Z","timestamp":1785422578437,"version":"3.56.0"},"reference-count":46,"publisher":"The Company of Biologists","license":[{"start":{"date-parts":[[2021,5,18]],"date-time":"2021-05-18T00:00:00Z","timestamp":1621296000000},"content-version":"am","delay-in-days":1598,"URL":"http:\/\/creativecommons.org\/licenses\/by\/3.0"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["RO1 HL118768"],"award-info":[{"award-number":["RO1 HL118768"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["K08 HL119553"],"award-info":[{"award-number":["K08 HL119553"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"abstract":"<jats:p>Originating as a single vessel emerging from the embryonic heart, the truncus arteriosus must septate and remodel into the aorta and pulmonary artery to support postnatal life. Defective remodeling or septation leads to abnormalities collectively known as conotruncal defects, which are associated with significant mortality and morbidity. Multiple populations of cells must interact to coordinate outflow tract remodeling, and cardiac neural crest has emerged as particularly important during this process. Abnormalities in cardiac neural crest have been implicated in the pathogenesis of multiple conotruncal defects, including persistent truncus arteriosus, double outlet right ventricle, and tetralogy of Fallot. However, the role of neural crest in the pathogenesis of another conotruncal abnormality, transposition of the great arteries, is less well understood. In this report, we demonstrate an unexpected role of Pdgfra in endothelial cells and their derivatives during outflow tract development. Loss of Pdgfra in endothelium and endothelial derived cells results in double outlet right ventricle and transposition of the great arteries. Our data suggest that loss of Pdgfra in endothelial-derived mesenchyme in the outflow tract endocardial cushions leads to a secondary defect in neural crest migration during development.<\/jats:p>","DOI":"10.1242\/dmm.029710","type":"journal-article","created":{"date-parts":[[2017,7,15]],"date-time":"2017-07-15T00:10:31Z","timestamp":1500077431000},"source":"Crossref","is-referenced-by-count":6,"title":["Pdgfr\u03b1 functions in endothelial-derived cells to regulate neural crest cells and development of the great arteries"],"prefix":"10.1242","author":[{"given":"Haig","family":"Aghajanian","sequence":"first","affiliation":[{"name":"Departments of Medicine & Cell and Developmental Biology, Penn Cardiovascular Institute, Institute for Regenerative Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Young Kuk","family":"Cho","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Chonnam National University Medical School. Gwangju, 61186, South Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicholas W.","family":"Rizer","sequence":"additional","affiliation":[{"name":"Departments of Medicine & Cell and Developmental Biology, Penn Cardiovascular Institute, Institute for Regenerative Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qiaohong","family":"Wang","sequence":"additional","affiliation":[{"name":"Departments of Medicine & Cell and Developmental Biology, Penn Cardiovascular Institute, Institute for Regenerative Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Li","family":"Li","sequence":"additional","affiliation":[{"name":"Departments of Medicine & Cell and Developmental Biology, Penn Cardiovascular Institute, Institute for Regenerative Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karl","family":"Degenhardt","sequence":"additional","affiliation":[{"name":"Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia, PA 19104, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1979-044X","authenticated-orcid":false,"given":"Rajan","family":"Jain","sequence":"additional","affiliation":[{"name":"Departments of Medicine & Cell and Developmental Biology, Penn Cardiovascular Institute, Institute for Regenerative Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"237","published-online":{"date-parts":[[2017,1,1]]},"reference":[{"key":"2021042612314645500_DMM029710C1","doi-asserted-by":"publisher","first-page":"948","DOI":"10.1136\/hrt.36.10.948","article-title":"Morphogenesis of bulboventricular malformations. 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