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Lookup NU author(s): Dr Ralf KistORCiD
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Heterozygous mutations in the human SOX9 gene cause campomelic dysplasia (CD), a skeletal malformation syndrome with various other organ defects. Severely affected CD patients usually die in the neonatal period due to respiratory distress. We analyzed the dynamic expression pattern of Sox9 in the developing mouse lung throughout morphogenesis. To determine a role of Sox9 in lung development and function, Sox9 was specifically inactivated in respiratory epithelial cells of the mouse lung using a doxycycline-inducible Cre/loxP system. Immunohistochemical and RNA analysis demonstrated extensive inactivation of Sox9 in the embryonic stage of lung development as early as embryonic day (E) 12.5. Lung morphogenesis and lung function after birth were not altered. Compensatory upregulation of Sox2, Sox4, Sox8, Sox10, Sox11, and Sox17 was not detected. Although Sox9 is expressed at high levels throughout lung morphogenesis, inactivation of Sox9 from the respiratory epithelial cells does not alter lung structure, postnatal survival, or repair following oxygen injury. © 2005 Wiley-Liss, Inc.
Author(s): Perl A-KT, Kist R, Shan Z, Scherer G, Whitsett JA
Publication type: Article
Publication status: Published
Journal: Genesis: The Journal of Genetics and Development
Year: 2005
Volume: 41
Issue: 1
Pages: 23-32
Print publication date: 01/01/2005
ISSN (print): 1526-954X
ISSN (electronic): 1526-968X
Publisher: Wiley
URL: http://dx.doi.org/10.1002/gene.20093
DOI: 10.1002/gene.20093
PubMed id: 15645446
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