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Normal lung development and function after Sox9 inactivation in the respiratory epithelium
Author(s) -
Perl AnneKarina T.,
Kist Ralf,
Shan Zhengyuan,
Scherer Gerd,
Whitsett Jeffrey A.
Publication year - 2005
Publication title -
genesis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.093
H-Index - 110
eISSN - 1526-968X
pISSN - 1526-954X
DOI - 10.1002/gene.20093
Subject(s) - respiratory epithelium , epithelium , respiratory system , lung , lung function , function (biology) , biology , microbiology and biotechnology , pathology , medicine , anatomy
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.