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Gosemann, JH; Doi, T; Kutasy, B; Friedmacher, F; Dingemann, J; Puri, P.
Pax3 gene expression is not altered during diaphragmatic development in nitrofen-induced congenital diaphragmatic hernia.
J Pediatr Surg. 2012; 47(6): 1067-1071. Doi: 10.1016/j.jpedsurg.2012.03.007
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Co-authors Med Uni Graz
Friedmacher Florian
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Abstract:
Malformations of the pleuroperitoneal folds (PPFs) have been identified as the origin of the diaphragmatic defect in congenital diaphragmatic hernia (CDH). Pax3, expressed in muscle precursor cells (MPCs), plays a key role in regulating myogenesis and muscularization in the fetal diaphragm. Pax3 mutant mice display absence of muscular diaphragm. However, the distribution of muscle precursor cells is reported to be normal in the PPF of the nitrofen-CDH model. We designed this study to investigate the hypothesis that Pax3 gene expression is unaltered in the PPF and developing diaphragm in the nitrofen-induced CDH model. Pregnant rats were treated with nitrofen or vehicle on gestational day (D) 9 and sacrificed on D13, D18, and D21. Pleuroperitoneal folds (D13) and developing diaphragms (D18 and D21) were dissected, total RNA was extracted, and real-time quantitative polymerase chain reaction was performed to determine Pax3 messenger RNA levels. Confocal immunofluorescence microscopy was performed to evaluate protein expression/distribution of Pax3. Relative messenger RNA expression levels of Pax3 in PPFs and developing diaphragms were not significantly different in the nitrofen group compared with controls. Intensity of Pax3 immunofluorescence was also not altered in PPFs and developing diaphragms of the nitrofen group compared with controls. Pax3 gene expression is not altered in the PPFs and developing diaphragm of nitrofen-CDH model, suggesting that the diaphragmatic defect is not caused by disturbance of myogenesis and muscularization. Copyright © 2012 Elsevier Inc. All rights reserved.
Find related publications in this database (using NLM MeSH Indexing)
Animals -
Diaphragm - embryology Diaphragm - metabolism Diaphragm - pathology
Embryonic Development - drug effects
Female -
Gene Expression Regulation, Developmental - drug effects
Hernia, Diaphragmatic - chemically induced Hernia, Diaphragmatic - embryology Hernia, Diaphragmatic - genetics Hernia, Diaphragmatic - metabolism
Hernias, Diaphragmatic, Congenital -
Microscopy, Fluorescence -
Paired Box Transcription Factors - biosynthesis Paired Box Transcription Factors - genetics
Peritoneum - embryology Peritoneum - metabolism Peritoneum - pathology
Phenyl Ethers - toxicity
Pleura - embryology Pleura - metabolism Pleura - pathology
Pregnancy -
RNA, Messenger - biosynthesis
Rats -
Rats, Sprague-Dawley -
Real-Time Polymerase Chain Reaction -

Find related publications in this database (Keywords)
Congenital diaphragmatic hernia
Nitrofen
Diaphragmatic development
Pax3
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