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Phenotypes Associated with This Genotype
either: 129.Cg-Tg(H2-K-Fosl2,EGFP)13Wag or B6.Cg-Tg(H2-K-Fosl2,EGFP)13Wag
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phenotype observed in females
phenotype observed in males
N normal phenotype
• mice die at 17 weeks of age with signs of respiratory distress

respiratory system
• at 17 weeks, mice exhibit pulmonary tissue stiffness
• end stage lungs exhibit fibrosis, dense lymphoid infiltration, hyperplasia of bronchus-associated lymphoid tissue and honeycombing of the peripheral lung lobules
• at 17 weeks
• mice exhibit secondary lung fibrosis that is preceded by pulmonary artery obliteration
• reconstituting mice with wild-type bone marrow or crossing mice to a Rag2 null background do not prevent fibrosis
• mice exhibit an increase in osteopontin, IL-2, IL-4, IL-6 and GM-CSF in the lungs with terminal staged lungs also expressing IL-1beta, IFN-gamma, CCL1, CCL2, CCL3, CCL4, CXCL2, CXCL10 and CXCL11
• however, lung fibrosis is not preceded by apoptosis
• at 17 weeks mice exhibit signs of respiratory distress such as tachypnea and hunched posture

cardiovascular system
• at 12 weeks, the pulmonary artery walls exhibit increased thickness compared to in wild-type mice
• obliteration of the pulmonary arteries, consisting of neointima formation induced by proliferation of vascular smooth muscle cells, coincides with perivascular inflammation
• mice exhibit fibrosis in the heart




• mice exhibit fibrosis in the skin

liver/biliary system
• mice exhibit fibrosis in the periportal tracts of the liver

renal/urinary system
• no fibrosis is observed in the kidney

endocrine/exocrine glands
• mice exhibit fibrosis in the thymus

hematopoietic system
• mice exhibit fibrosis in the thymus

immune system
• mice exhibit fibrosis in the thymus

digestive/alimentary system
• mice exhibit fibrosis in the esophagus
• mice exhibit fibrosis in the stomach

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
idiopathic pulmonary fibrosis DOID:0050156 OMIM:178500

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
MGI 6.12
The Jackson Laboratory