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

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

neoplasm

increased malignant tumor incidence ( J:204282 )

• 2 of 5 mutants that develop ascites after 4 months of doxycycline induction contain cells with malignant cytology

increased blastoma incidence ( J:224791 )

• 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

increased lung tumor incidence ( J:204282 )

• 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

increased lung adenoma incidence ( J:204282 )

• after 4 days of doxycycline induction, nodules are histologically consistent with papillary adenoma

increased lung adenocarcinoma incidence ( J:204282 )

• after 8 days of doxycycline induction, the cells forming papillary structures become atypical with larger and hyperchromatic nuclei, consistent with papillary adenocarcinoma

cellular

increased mesenchymal cell proliferation involved in lung development ( J:224791 )

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

mortality/aging

premature death ( J:204282 )

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

respiratory system

increased mesenchymal cell proliferation involved in lung development ( J:224791 )

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

increased lung tumor incidence ( J:204282 )

• 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

increased lung adenoma incidence ( J:204282 )

• after 4 days of doxycycline induction, nodules are histologically consistent with papillary adenoma

increased lung adenocarcinoma incidence ( J:204282 )

• after 8 days of doxycycline induction, the cells forming papillary structures become atypical with larger and hyperchromatic nuclei, consistent with papillary adenocarcinoma

abnormal branching involved in lung morphogenesis ( J:224791 )

• lungs exhibit reduced branching after doxycycline induction from E10.5 to E12.5

abnormal lung-associated mesenchyme development ( J:224791 )

• lungs exhibit expanded mesenchyme after doxycycline induction from E10.5 to E12.5

abnormal lung epithelium morphology ( J:224791 )

• lungs exhibit dilated epithelial ducts after doxycycline induction from E10.5 to E12.5

respiratory failure ( J:204282 )

• doxycycline treated adults die due to respiratory failure

homeostasis/metabolism

ascites ( J:204282 )

• mutants surviving 4 months of doxycycline induction develop ascites

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
lung cancer		DOID:1324	OMIM:211980 OMIM:608935 OMIM:612571 OMIM:612593 OMIM:614210	J:204282
pleuropulmonary blastoma		DOID:4769		J:224791