Toggle Main Menu Toggle Search

Open Access padlockePrints

Normal lung development and function after Sox9 inactivation in the respiratory epithelium

Lookup NU author(s): Dr Ralf KistORCiD


Full text for this publication is not currently held within this repository. Alternative links are provided below where available.


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.

Publication metadata

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


DOI: 10.1002/gene.20093

PubMed id: 15645446


Altmetrics provided by Altmetric


Funder referenceFunder name