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Phenotypes Associated with This Genotype
Genotype
MGI:5538520
Allelic
Composition
Tg(SFTPC-rtTA)5Jaw/0
Tg(tetO-Fgf9,-EGFP)#Dor/0
Genetic
Background
involves: FVB
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(SFTPC-rtTA)5Jaw mutation (3 available)
Tg(tetO-Fgf9,-EGFP)#Dor mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
neoplasm
• 2 of 5 mutants that develop ascites after 4 months of doxycycline induction contain cells with malignant cytology
• doxycycline induction from E16.5 to E18.5 results in lung mesenchymal hyperplasia beneath a benign-appearing Nkx2.1 positive epithelium with histological features of Type I or cystic pleuropulmonary blastoma
• one day following doxycycline administration, adult mutants exhibit formation of small nodules (epithelial clusters) on the lung surface
• one day following doxycycline administration, adult mutants exhibit formation of small nodules (epithelial clusters) on the lung surface
• papillary adenoma and papillary adenocarcinoma develop in mice treated with doxycycline for 4 and 8 days, respectively
• small tumor nodules and large tumor nodules persist in the lungs of mutants treated with doxycycline for 2-4 days followed by 1 month of doxycycline and in mutants treated with doxycycline for 2 weeks followed by 9 and 18 weeks of withdrawal, respectively
• after 4 days of doxycycline induction, nodules are histologically consistent with papillary adenoma
• after 8 days of doxycycline induction, the cells forming papillary structures become atypical with larger and hyperchromatic nuclei, consistent with papillary adenocarcinoma

cellular
• lungs show increased mesenchymal proliferation after doxycycline induction from E10.5 to E12.5

mortality/aging
• doxycycline treated adults show a median survival of 89 days and die due to respiratory failure

respiratory system
• lungs show increased mesenchymal proliferation after doxycycline induction from E10.5 to E12.5
• one day following doxycycline administration, adult mutants exhibit formation of small nodules (epithelial clusters) on the lung surface
• one day following doxycycline administration, adult mutants exhibit formation of small nodules (epithelial clusters) on the lung surface
• papillary adenoma and papillary adenocarcinoma develop in mice treated with doxycycline for 4 and 8 days, respectively
• small tumor nodules and large tumor nodules persist in the lungs of mutants treated with doxycycline for 2-4 days followed by 1 month of doxycycline and in mutants treated with doxycycline for 2 weeks followed by 9 and 18 weeks of withdrawal, respectively
• after 4 days of doxycycline induction, nodules are histologically consistent with papillary adenoma
• after 8 days of doxycycline induction, the cells forming papillary structures become atypical with larger and hyperchromatic nuclei, consistent with papillary adenocarcinoma
• lungs exhibit reduced branching after doxycycline induction from E10.5 to E12.5
• lungs exhibit expanded mesenchyme after doxycycline induction from E10.5 to E12.5
• lungs exhibit dilated epithelial ducts after doxycycline induction from E10.5 to E12.5
• doxycycline treated adults die due to respiratory failure

homeostasis/metabolism
• mutants surviving 4 months of doxycycline induction develop ascites

Mouse Models of Human Disease
OMIM ID Ref(s)
Lung Cancer 211980 J:204282
Pleuropulmonary Blastoma; PPB 601200 J:224791


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
06/15/2016
MGI 6.04
The Jackson Laboratory