Mesenchymal expression of the FRAS1/FREM2 gene unit is decreased in the developing fetal diaphragm of nitrofen-induced congenital diaphragmatic hernia

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• 作者：Toshiaki Takahashi ; Florian Friedmacher ; Julia Zimmer…
• 关键词：FRAS1 ; FREM2 ; Fraser syndrome ; Diaphragm ; Congenital diaphragmatic hernia ; Nitrofen
• 刊名：Pediatric Surgery International
• 出版年：2016
• 出版时间：February 2016
• 年：2016
• 卷：32
• 期：2
• 页码：135-140
• 全文大小：755 KB
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• 作者单位：Toshiaki Takahashi (1)
  Florian Friedmacher (1)
  Julia Zimmer (1)
  Prem Puri (1) (2)
  
  1. National Children’s Research Centre, Our Lady’s Children’s Hospital, Crumlin, Dublin 12, Ireland
  2. Conway Institute of Biomolecular and Biomedical Research, School of Medicine and Medical Science, University College Dublin, Dublin, Ireland
  
• 刊物类别：Medicine
• 刊物主题：Medicine & Public Health
  Pediatrics
  Surgery
  Pediatric Surgery
  
• 出版者：Springer Berlin / Heidelberg
• ISSN：1437-9813

文摘

Purpose Developmental mutations that inhibit normal formation of extracellular matrix (ECM) in fetal diaphragms have been identified in congenital diaphragmatic hernia (CDH). FRAS1 and FRAS1-related extracellular matrix 2 (FREM2), which encode important ECM proteins, are secreted by mesenchymal cells during diaphragmatic development. The FRAS1/FREM2 gene unit has been shown to form a ternary complex with FREM1, which plays a crucial role during formation of human and rodent diaphragms. Furthermore, it has been demonstrated that the diaphragmatic expression of FREM1 is decreased in the nitrofen-induced CDH model. We hypothesized that FRAS1 and FREM2 expression is decreased in the developing diaphragms of fetal rats with nitrofen-induced CDH.