Mouse Genome Informatics
tg
    Tg(Lck-Tnfsf4)1Nish/0
B6.Cg-Tg(Lck-Tnfsf4)1Nish
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
immune system
• Background Sensitivity: an increase in the ratio of effector/memory T cells to regulatory T cells is seen in the lung on the C57BL/6 background but not on the BALB/c background
• increase in the number of effector/memory CD4+ T cells in the spleen and lungs
• Background Sensitivity: the number of IL-17 producing CD4+ T cells in response to PMA/ionomycin stimulation is increased in 8-10 week old mutants; this increase is greater on the C57BL/6 background than on the BABL/c background
• Background Sensitivity: the frequency and absolute number of T regulatory cells is increased at 8-10 weeks of age; this increase is less on the C57BL/6 than on the BALB/c background
• increased hapten-specific T cell proliferative response
• Background Sensitivity: IL-13 production by CD4+ T cells in the lungs in response to anti-CD3 stimulation is increased in 8-10 week old mice; this increase is greater on the C57BL/6 background than on the BALB/c background
• increased ear swelling 24 hours following challenge with 4-dinitrofluorobenzene (DNFB), 6-trinitrochlorobenzene (TNCB), and FITC compared to wild-type
• increased hapten-specific cytokine production
• Background Sensitivity: IL-13 production by CD4+ T cells in the lungs in response to anti-CD3 stimulation is increased in 8-10 week old mice; this increase is greater on the C57BL/6 background than on the BALB/c background
• vascular lesions are accompanied with perivascular infiltration of lymphocytes and, to a lesser extent, neutrophils
• lesions and inflammation are seen mainly in arteries contained in bronchovascular bundles (respiratory arteries)
• perivascular lymphocytic infiltration is seen around pulmonary veins, however no remodeling is seen in those veins

hematopoietic system
• Background Sensitivity: an increase in the ratio of effector/memory T cells to regulatory T cells is seen in the lung on the C57BL/6 background but not on the BALB/c background
• increase in the number of effector/memory CD4+ T cells in the spleen and lungs
• Background Sensitivity: the number of IL-17 producing CD4+ T cells in response to PMA/ionomycin stimulation is increased in 8-10 week old mutants; this increase is greater on the C57BL/6 background than on the BABL/c background
• Background Sensitivity: the frequency and absolute number of T regulatory cells is increased at 8-10 weeks of age; this increase is less on the C57BL/6 than on the BALB/c background
• increased hapten-specific T cell proliferative response
• Background Sensitivity: IL-13 production by CD4+ T cells in the lungs in response to anti-CD3 stimulation is increased in 8-10 week old mice; this increase is greater on the C57BL/6 background than on the BALB/c background

cardiovascular system
• Background Sensitivity: mice on the C57BL/6, but not BALB/c, background exhibit diffuse pathological changes in the small- to medium-sized pulmonary arteries, indicating formation of vascular lesions in the lungs
• lesions and inflammation are seen mainly in arteries contained in bronchovascular bundles (respiratory arteries)
• mice exhibit fibrocellular endothelial proliferation of the intimal layer and, to a lesser extent, medial muscular hypertrophy
• however, muscularization of distal pulmonary arterioles and plexiform lesions are not seen
• no vascular lesions are seen in the kidney or colon
• Background Sensitivity: right ventricular hypertrophy is seen on the C57BL/6, but not BALB/c, background
• Background Sensitivity: vasoconstriction is frequently seen in pulmonary arteries of mice on the C57BL/6, but not BALB/c, background
• Background Sensitivity: right ventricular systolic pressure is increased in aged mice on the C57BL/6, but not BALB/c, background

muscle
• Background Sensitivity: vasoconstriction is frequently seen in pulmonary arteries of mice on the C57BL/6, but not BALB/c, background

respiratory system
• vascular lesions are accompanied with perivascular infiltration of lymphocytes and, to a lesser extent, neutrophils
• lesions and inflammation are seen mainly in arteries contained in bronchovascular bundles (respiratory arteries)
• perivascular lymphocytic infiltration is seen around pulmonary veins, however no remodeling is seen in those veins
• Background Sensitivity: mice on the C57BL/6, but not BALB/c, background develop spontaneous lung disease characterized by lung-specific, pulmonary artery-restricted intimal thickening with lymphocytic chronic inflammation

Mouse Models of Human Disease
OMIM IDRef(s)
Pulmonary Hypertension, Primary, 1; PPH1 178600 J:194882