Phenotypes Associated with This Genotype

Genotype
MGI:3813506

• Allelic Composition: Tg(H2-K-Fosl2,-EGFP)13Wag/0
• Genetic Background: either: 129.Cg-Tg(H2-K-Fosl2,-EGFP)13Wag or B6.Cg-Tg(H2-K-Fosl2,-EGFP)13Wag

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

mortality/aging

premature death ( J:139032 )

• mice die at 17 weeks of age with signs of respiratory distress

respiratory system

abnormal lung morphology ( J:139032 )

• 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

increased lung weight ( J:139032 )

• at 17 weeks

pulmonary fibrosis ( J:139032 )

• 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

tachypnea ( J:139032 )

respiratory distress ( J:139032 )

• at 17 weeks mice exhibit signs of respiratory distress such as tachypnea and hunched posture

cardiovascular system

abnormal pulmonary artery morphology ( J:139032 )

• 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

cardiac fibrosis ( J:139032 )

• mice exhibit fibrosis in the heart

skeleton

increased bone mass ( J:139032 )

behavior/neurological

hunched posture ( J:139032 )

neoplasm

increased fibrosarcoma incidence ( J:139032 )

• rarely

integument

skin fibrosis ( J:139032 )

• mice exhibit fibrosis in the skin

liver/biliary system

liver fibrosis ( J:139032 )

• mice exhibit fibrosis in the periportal tracts of the liver

renal/urinary system

renal/urinary system phenotype ( J:139032 )

N • no fibrosis is observed in the kidney

endocrine/exocrine glands

thymus fibrosis ( J:139032 )

• mice exhibit fibrosis in the thymus

hematopoietic system

thymus fibrosis ( J:139032 )

• mice exhibit fibrosis in the thymus

immune system

thymus fibrosis ( J:139032 )

• mice exhibit fibrosis in the thymus

digestive/alimentary system

esophagus fibrosis ( J:139032 )

• mice exhibit fibrosis in the esophagus

stomach fibrosis ( J:139032 )

• mice exhibit fibrosis in the stomach

growth/size/body

increased lung weight ( J:139032 )

• at 17 weeks

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
idiopathic pulmonary fibrosis		DOID:0050156	OMIM:178500	J:139032