Journal of Pediatric Surgery 2012-06-01

Pax3gene expression is not altered during diaphragmatic development in nitrofen-induced congenital diaphragmatic hernia

Jan-Hendrik Gosemann, Takashi Doi, Balazs Kutasy, Florian Friedmacher, Jens Dingemann, Prem Puri, Jan-Hendrik Gosemann, Takashi Doi, Balazs Kutasy, Florian Friedmacher, Jens Dingemann, Prem Puri

Index: J. Pediatr. Surg. 47(6) , 1067-71, (2012)

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Abstract

Background/Purpose 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.