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Publication : A genetic model for a central (septum transversum) congenital diaphragmatic hernia in mice lacking Slit3.

First Author  Yuan W Year  2003
Journal  Proc Natl Acad Sci U S A Volume  100
Issue  9 Pages  5217-22
PubMed ID  12702769 Mgi Jnum  J:83292
Mgi Id  MGI:2660972 Doi  10.1073/pnas.0730709100
Citation  Yuan W, et al. (2003) A genetic model for a central (septum transversum) congenital diaphragmatic hernia in mice lacking Slit3. Proc Natl Acad Sci U S A 100(9):5217-22
abstractText  Congenital diaphragmatic hernia (CDH) is a significant cause of pediatric mortality in humans with a heterogeneous and poorly understood etiology. Here we show that mice lacking Slit3 developed a central (septum transversum) CDH. Slit3 encodes a member of the Slit family of guidance molecules and is expressed predominantly in the mesothelium of the diaphragm during embryonic development. In Slit3 null mice, the central tendon region of the diaphragm fails to separate from liver tissue because of abnormalities in morphogenesis. The CDH progresses through continuous growth of the liver into the thoracic cavity. This study establishes the first genetic model for CDH and identifies a previously unsuspected role for Slit3 in regulating the development of the diaphragm.
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