Decreased Endoglin expression in the pulmonary vasculature of nitrofen-induced congenital diaphragmatic hernia rat model

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• 作者：Julia Zimmer ; Toshiaki Takahashi ; Alejandro D. Hofmann…
• 关键词：Congenital diaphragmatic hernia ; Nitrofen ; Pulmonary hypertension ; Endoglin ; Tgf beta signalling
• 刊名：Pediatric Surgery International
• 出版年：2017
• 出版时间：February 2017
• 年：2017
• 卷：33
• 期：2
• 页码：263-268
• 全文大小：
• 刊物类别：Medicine
• 刊物主题：Pediatrics; Surgery; Pediatric Surgery;
• 出版者：Springer Berlin Heidelberg
• ISSN：1437-9813
• 卷排序：33

文摘

Aim of the StudyPulmonary hypertension (PH) remains a therapeutical challenge in neonates born with congenital diaphragmatic hernia (CDH). Endoglin (Eng), an auxiliary receptor component of the transforming growth factor β (TGFβ) signalling pathway, is expressed mainly by endothelial cells and has been found to be involved in angiogenesis and vascular remodelling. Genetic studies have linked TGFβ and Eng mutations to human arterial PH and other cardiovascular syndromes. Eng interacts with the TGFβ receptors 1 and 2 (Tgfβr1, Tgfβr2). We designed this study to investigate the hypothesis that Eng is altered in the pulmonary vasculature of rats with nitrofen-induced CDH subjected to its interdependency with Tgfβr1 and Tgfβr2.