AJP - Lung Watch the video to learn how APS reaches out to developing nations.
HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
QUICK SEARCH:   [advanced]


     

Am J Physiol Lung Cell Mol Physiol 283: L1310-L1314, 2002. First published August 23, 2002; doi:10.1152/ajplung.00257.2002
1040-0605/02 $5.00
This Article
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow All Versions of this Article:
283/6/L1310    most recent
00257.2002v1
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in Web of Science
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Web of Science (31)
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Babiuk, R. P.
Right arrow Articles by Greer, J. J.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Babiuk, R. P.
Right arrow Articles by Greer, J. J.
Vol. 283, Issue 6, L1310-L1314, December 2002

Diaphragm defects occur in a CDH hernia model independently of myogenesis and lung formation

Randal P. Babiuk and John J. Greer

Department of Physiology, Perinatal Research Centre, 513 Heritage Medical Research Centre, University of Alberta, Edmonton, Alberta, Canada T6G 2S2

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 because of 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.

c-met; hepatocyte growth factor/scatter factor; Fgf10; somatopleure; neonatology; congenital diaphragmatic hernia


This article has been cited by other articles:


Home page
 J. Appl. Physiol.Home page
C. B. Mantilla and G. C. Sieck
Key aspects of phrenic motoneuron and diaphragm muscle development during the perinatal period
J Appl Physiol, June 1, 2008; 104(6): 1818 - 1827.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Physiol. Lung Cell. Mol. Physiol.Home page
R. D. Clugston, W. Zhang, and J. J. Greer
Gene expression in the developing diaphragm: significance for congenital diaphragmatic hernia
Am J Physiol Lung Cell Mol Physiol, April 1, 2008; 294(4): L665 - L675.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Physiol. Lung Cell. Mol. Physiol.Home page
J. R. Bourbon and A. Benachi
CFTR gene therapy, a method to rescue lung hypoplasia in congenital diaphragmatic hernia?
Am J Physiol Lung Cell Mol Physiol, July 1, 2006; 291(1): L1 - L3.
[Full Text] [PDF]

Home page
 Am. J. Physiol. Lung Cell. Mol. Physiol.Home page
R. P. Babiuk, B. Thebaud, and J. J. Greer
Reductions in the incidence of nitrofen-induced diaphragmatic hernia by vitamin A and retinoic acid
Am J Physiol Lung Cell Mol Physiol, May 1, 2004; 286(5): L970 - L973.
[Abstract] [Full Text] [PDF]


HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
Visit Other APS Journals Online