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Am J Physiol Lung Cell Mol Physiol (August 23, 2002). doi:10.1152/ajplung.00257.2002
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Articles in PresS, published online ahead of print August 23, 2002
Am J Physiol Lung Cell Mol Physiol, 10.1152/ajplung.00257.2002
Submitted on August 7, 2002
Accepted on August 14, 2002

Diaphragm defects occur in a congenital diaphragmatic hernia model independent of myogenesis and lung formation

Randal P. Babiuk1 and John J. Greer1*

1 Department of Physiology, University of Alberta, Edmonton, AB, none

* To whom correspondence should be addressed. E-mail: John.greer{at}ualberta.com.

Congenital diaphragmatic hernia (CDH) is a significant clinical problem in which a portion of the diaphragmatic musculature fails to form, resulting in a hole in the diaphragm. Here we use animal models of CDH to test two hypotheses regarding the pathogenesis. First, the origin of the defect results from the malformation of the amuscular mesenchymal component of the primordial diaphragm rather than with the process of myogenesis. Second, the defect in the primordial diaphragmatic tissue is not secondary to defects in the developing lung. In c-met-/- mouse embryos, in which diaphragm muscle fibers do not form due to a defect in muscle precursor migration, the amuscular substratum forms fully. We show that a defect characteristic of CDH can be induced in the amuscular membrane. In Fgf10-/- mouse embryos, that have lung agenesis, we show that the primordial diaphragm does not depend on signals from lung tissue for proper development and that diaphragmatic malformation is a primary defect in CDH. These data suggest that the pathogenesis of CDH involves mechanisms fundamentally different from previously proposed hypotheses.




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