Mouse Genome Informatics
either: 129.Cg-Tg(H2-K-Fosl2,EGFP)13Wag or B6.Cg-Tg(H2-K-Fosl2,EGFP)13Wag
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
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 lungs, skin, thymus and the periportal tracts of the liver, stomach, esophagus and heart
• however, no fibrosis is observed in the kidney

Mouse Models of Human Disease
Pulmonary Fibrosis, Idiopathic; IPF 178500 J:139032