A.-K.T. Perl and R. Kist contributed equally to this work.
Normal lung development and function after Sox9 inactivation in the respiratory epithelium
Version of Record online: 11 JAN 2005
Copyright © 2005 Wiley-Liss, Inc.
Volume 41, Issue 1, pages 23–32, January 2005
How to Cite
Perl, A.-K. T., Kist, R., Shan, Z., Scherer, G. and Whitsett, J. A. (2005), Normal lung development and function after Sox9 inactivation in the respiratory epithelium. Genesis, 41: 23–32. doi: 10.1002/gene.20093
- Issue online: 11 JAN 2005
- Version of Record online: 11 JAN 2005
- Manuscript Accepted: 25 SEP 2004
- Manuscript Received: 14 JUL 2004
- National Institutes of Health. Grant Number: HL 56387
- Deutsche Forschungsgemeinschaft. Grant Number: Sche 194/15-1
- conditional inactivation;
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. genesis 41:23–32, 2005. © 2005 Wiley-Liss, Inc.