Phenotypes Associated with This Genotype

Genotype
MGI:5927436

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

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

respiratory system

abnormal lung vasculature morphology ( J:238723 )

• beginning at 10 weeks of age, mice develop pulmonary arterial vascular remodeling, characterized by increased wall thickness and narrowing of the arterial lumen

• mice treated with the pan-TGFbeta-neutralizing monoclonal antibody 1D11 show amelioration of the pulmonary vascular remodeling

lung inflammation ( J:238723 )

• mice develop pulmonary inflammation, most prominently around the distal vasculature and airways starting at 6-10 weeks of age, with increases in macrophages, lymphocytes, and especially, granulocytes in BAL fluid

• pulmonary fibrosis is prominent in the perivascular area but also seen in the peripheral, subpleural lung

• mice treated with the pan-TGFbeta-neutralizing monoclonal antibody 1D11 show increased severity of lung inflammation

pulmonary fibrosis ( J:238723 )

• mice develop pulmonary fibrosis that becomes prominent by 14 weeks of age

• hydroxyproline content in the lungs is increased at 14 weeks of age

• treatment with the pan-TGFbeta-neutralizing monoclonal antibody 1D11 has no effect on pulmonary fibrosis

cardiovascular system

abnormal lung vasculature morphology ( J:238723 )

• beginning at 10 weeks of age, mice develop pulmonary arterial vascular remodeling, characterized by increased wall thickness and narrowing of the arterial lumen

• mice treated with the pan-TGFbeta-neutralizing monoclonal antibody 1D11 show amelioration of the pulmonary vascular remodeling

vascular smooth muscle hyperplasia ( J:238723 )

• expansion of the vascular smooth muscle compartment in peripheral pulmonary arteries of lungs

• increase in the proportion of Ki67-positive smooth muscle cells in the lungs, indicating increased proliferation

immune system

lung inflammation ( J:238723 )

• mice develop pulmonary inflammation, most prominently around the distal vasculature and airways starting at 6-10 weeks of age, with increases in macrophages, lymphocytes, and especially, granulocytes in BAL fluid

• pulmonary fibrosis is prominent in the perivascular area but also seen in the peripheral, subpleural lung

• mice treated with the pan-TGFbeta-neutralizing monoclonal antibody 1D11 show increased severity of lung inflammation

muscle

vascular smooth muscle hyperplasia ( J:238723 )

• expansion of the vascular smooth muscle compartment in peripheral pulmonary arteries of lungs

• increase in the proportion of Ki67-positive smooth muscle cells in the lungs, indicating increased proliferation

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
systemic scleroderma		DOID:418	OMIM:181750	J:238723