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Aberrant Bmp signaling and notochord delamination in the pathogenesis of esophageal atresia.

Authors: Li, Yina  Litingtung, Ying  Ten Dijke, Peter  Chiang, Chin 
Citation: Li Y, etal., Dev Dyn. 2007 Mar;236(3):746-54.
Pubmed: (View Article at PubMed) PMID:17260385
DOI: Full-text: DOI:10.1002/dvdy.21075

Human foregut malformation known as esophageal atresia with tracheoesophageal fistula (EA/TEF) occurs in 1 in 4,000 live births with unknown etiology. We found that mice lacking Noggin (Nog(-/-)) displayed Type C EA/TEF, the most common form in humans, and notochordal defects strikingly similar to the adriamycin-induced rat EA/TEF model. In accord with esophageal atresia, Nog(-/-) embryos displayed reduction in the dorsal foregut endoderm, which was associated with reduced adhesion and disrupted basement membrane. However, significant apoptosis in the Nog(-/-) dorsal foregut was not observed. Instead, non-notochordal, likely endodermal, cells were found in Nog(-/-) notochord, suggesting that Noggin function is required in the notochordal plate for its proper delamination from the dorsal foregut. Notably, ablating Bmp7 function in Nog(-/-) embryos rescued EA/TEF and notochord branching defects, establishing a critical role of Noggin-mediated Bmp7 antagonism in EA/TEF pathogenesis.

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CRRD Object Information
CRRD ID: 12801454
Created: 2017-03-31
Species: All species
Last Modified: 2017-03-31
Status: ACTIVE



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RGD is funded by grant HL64541 from the National Heart, Lung, and Blood Institute on behalf of the NIH.