{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,11]],"date-time":"2026-09-11T19:05:21Z","timestamp":1789153521256,"version":"build-2803163510"},"reference-count":22,"publisher":"S. Karger AG","issue":"4","license":[{"start":{"date-parts":[[2024,1,1]],"date-time":"2024-01-01T00:00:00Z","timestamp":1704067200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/karger.com\/pages\/terms-and-conditions"},{"start":{"date-parts":[[2024,1,1]],"date-time":"2024-01-01T00:00:00Z","timestamp":1704067200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/karger.com\/pages\/terms-and-conditions"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neonatology"],"published-print":{"date-parts":[[2024]]},"abstract":"<b><i>Introduction:<\/i><\/b> Congenital diaphragmatic hernia (CDH) is a complex congenital disorder, characterized by pulmonary hypertension (PH) and hypoplasia. PH secondary to CDH (CDH-PH) features devastating morbidity and mortality (25\u201330%) among neonates. An unmet need is determining mechanisms triggering CDH-PH to save infants. Prior data suggest abnormal remodeling of the pulmonary vascular extracellular matrix (ECM), presumed to be driven by endothelial-to-mesenchymal transition (EndoMT), hinders postnatal vasodilation and limits anti-PH therapy in CDH. There are limited data on the role of EndoMT in CDH-PH. <b><i>Methods:<\/i><\/b> The purpose of the study was to investigate how EndoMT contributes to CDH-PH by identifying cells undergoing EndoMT noted by alpha smooth muscle actin (\u03b1-SMA) expression in human umbilical vein endothelial cells (HUVECs) and lung tissue obtained from murine pups using the nitrofen model. <i>N<\/i> = 8 CDH, <i>N<\/i> = 8 control HUVECs were stained for \u03b1-SMA and CD31 after being exposed for 24 h to TGFB, a known EndoMT promoter. <i>N<\/i> = 8 nitrofen, <i>N<\/i> = 8 control murine pup lungs were also stained for \u03b1-SMA and CD31. \u03b1-SMA and CD31 expression was quantified in HUVECs and murine tissue using Fiji imaging software and normalized to the total number of cells per slide noted by DAPI staining. <b><i>Results:<\/i><\/b> CDH HUVECs demonstrated a 1.1-fold increase in \u03b1-SMA expression (<i>p<\/i> = 0.02). The murine model did not show statistical significance between nitrofen and control pup lungs; however, there was a 0.4-fold increase in \u03b1-SMA expression with a 0.8-fold decrease in CD31 expression in the nitrofen pup lungs when compared to controls. <b><i>Conclusion:<\/i><\/b> These results suggest that EndoMT could potentially play a role in the ECM remodeling seen in CDH-PH. <\/jats:p>","DOI":"10.1159\/000537802","type":"journal-article","created":{"date-parts":[[2024,4,8]],"date-time":"2024-04-08T21:00:31Z","timestamp":1712610031000},"page":"512-518","source":"Crossref","is-referenced-by-count":2,"title":["Endothelial-to-Mesenchymal Transition in Human and Murine Models of Congenital Diaphragmatic Hernia"],"prefix":"10.1159","volume":"121","author":[{"given":"Jamie","family":"Gilley","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sandra K.","family":"Hanneman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Madelene J.","family":"Ottosen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Binoy","family":"Shivanna","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sundeep","family":"Keswani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"127","published-online":{"date-parts":[[2024,4,8]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","DOI":"10.1002\/bdr2.1106"},{"key":"ref2","doi-asserted-by":"publisher","DOI":"10.5144\/0256-4947.2006.391"},{"key":"ref3","doi-asserted-by":"publisher","DOI":"10.1053\/j.semperi.2019.07.006"},{"key":"ref4","doi-asserted-by":"publisher","DOI":"10.1186\/s12931-0170670-2"},{"key":"ref5","doi-asserted-by":"publisher","DOI":"10.1016\/j.siny.2022.10136"},{"key":"ref6","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpedsurg.2010.12.015"},{"key":"ref7","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.24914"},{"key":"ref8","doi-asserted-by":"publisher","DOI":"10.1016\/s0022-3468(97)90587-x"},{"key":"ref9","doi-asserted-by":"publisher","DOI":"10.3760\/cma.j.issn.0529-5815.2018.11.018"},{"key":"ref10","doi-asserted-by":"publisher","DOI":"10.1161\/ATVBAHA.115.305887"},{"key":"ref11","doi-asserted-by":"publisher","DOI":"10.1038\/srep33787"},{"key":"ref12","doi-asserted-by":"publisher","DOI":"10.1038\/s41390-019-0345-4"},{"key":"ref13","doi-asserted-by":"publisher","DOI":"10.3390\/app10030938"},{"key":"ref14","doi-asserted-by":"publisher","DOI":"10.1038\/nprot.2007.54"},{"key":"ref15","doi-asserted-by":"publisher","DOI":"10.1038\/ncomms11853"},{"key":"ref16","doi-asserted-by":"publisher","DOI":"10.1055\/s-0038-1676830"},{"key":"ref17","doi-asserted-by":"publisher","DOI":"10.3389\/fped.2014.00036"},{"key":"ref18","doi-asserted-by":"publisher","DOI":"10.1038\/s41390-020-01191-x"},{"key":"ref19","doi-asserted-by":"publisher","DOI":"10.1152\/ajplung.1998.274.4.L636"},{"key":"ref20","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajpath.2011.06.001"},{"key":"ref21","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajpath.2013.05.019"},{"key":"ref22","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-018-29153-8"}],"container-title":["Neonatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/karger.com\/neo\/article-pdf\/121\/4\/512\/4264211\/000537802.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/karger.com\/neo\/article-pdf\/121\/4\/512\/4264211\/000537802.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,23]],"date-time":"2025-04-23T15:06:40Z","timestamp":1745420800000},"score":1,"resource":{"primary":{"URL":"https:\/\/karger.com\/article\/doi\/10.1159\/000537802"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024]]},"references-count":22,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2024,8,9]]},"published-print":{"date-parts":[[2024,8,14]]}},"URL":"https:\/\/doi.org\/10.1159\/000537802","archive":["Portico"],"relation":{},"ISSN":["1661-7800","1661-7819"],"issn-type":[{"value":"1661-7800","type":"print"},{"value":"1661-7819","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024]]}}}