Phenotypes associated with this allele

• Allele Symbol: Tg(Lck-Tnfsf4)1Nish
• Allele Name: transgene insertion 1, Naoto Ishii
• Allele ID: MGI:2388725
• Summary: 2 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
tg1	Tg(Lck-Tnfsf4)1Nish/0	B6.Cg-Tg(Lck-Tnfsf4)1Nish	MGI:2651594
tg2	Tg(Lck-Tnfsf4)1Nish/0	C.Cg-Tg(Lck-Tnfsf4)1Nish	MGI:5475021

Genotype
MGI:2651594

tg1

• Allelic Composition: Tg(Lck-Tnfsf4)1Nish/0
• Genetic Background: B6.Cg-Tg(Lck-Tnfsf4)1Nish

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

immune system

increased T cell proliferation ( J:80214 )

• increased hapten-specific T cell proliferative response

abnormal T cell subpopulation ratio ( J:194882 )

• 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

increased CD4-positive, alpha-beta T cell number ( J:194882 )

• 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

increased regulatory T cell number ( J:194882 )

• 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

abnormal CD4-positive, alpha-beta T cell physiology ( J:194882 )

• 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 susceptibility to type IV hypersensitivity reaction ( J:80214 )

• increased ear swelling 24 hours following challenge with 4-dinitrofluorobenzene (DNFB), 6-trinitrochlorobenzene (TNCB), and FITC compared to wild-type

abnormal cytokine secretion ( J:80214 )

• increased hapten-specific cytokine production

increased interleukin-13 secretion ( J:194882 )

• 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

lung inflammation ( J:194882 )

• 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

increased T cell proliferation ( J:80214 )

• increased hapten-specific T cell proliferative response

abnormal T cell subpopulation ratio ( J:194882 )

• 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

increased CD4-positive, alpha-beta T cell number ( J:194882 )

• 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

increased regulatory T cell number ( J:194882 )

• 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

abnormal CD4-positive, alpha-beta T cell physiology ( J:194882 )

• 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

abnormal pulmonary artery morphology ( J:194882 )

• 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

heart right ventricle hypertrophy ( J:194882 )

• Background Sensitivity: right ventricular hypertrophy is seen on the C57BL/6, but not BALB/c, background

increased right ventricle systolic pressure ( J:194882 )

• Background Sensitivity: right ventricular systolic pressure is increased in aged mice on the C57BL/6, but not BALB/c, background

increased vasoconstriction ( J:194882 )

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

muscle

heart right ventricle hypertrophy ( J:194882 )

• Background Sensitivity: right ventricular hypertrophy is seen on the C57BL/6, but not BALB/c, background

increased vasoconstriction ( J:194882 )

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

respiratory system

lung inflammation ( J:194882 )

• 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

abnormal lung morphology ( J:194882 )

• 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

growth/size/body

heart right ventricle hypertrophy ( J:194882 )

• Background Sensitivity: right ventricular hypertrophy is seen on the C57BL/6, but not BALB/c, background

cellular

increased T cell proliferation ( J:80214 )

• increased hapten-specific T cell proliferative response

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
primary pulmonary hypertension		DOID:14557	OMIM:178600 OMIM:265400 OMIM:615342 OMIM:615343 OMIM:615344	J:194882

Genotype
MGI:5475021

tg2

• Allelic Composition: Tg(Lck-Tnfsf4)1Nish/0
• Genetic Background: C.Cg-Tg(Lck-Tnfsf4)1Nish

cardiovascular system

cardiovascular system phenotype ( J:194882 )

N • Background Sensitivity: mice on the BALB/c background do not develop vascular lesions in the lungs, vasoconstriction in pulmonary arteries, an increase in right ventricular systolic pressure, or right ventricle hypertrophy as seen in mice on a C57BL/6 background

hematopoietic system

increased CD4-positive, alpha-beta T cell number ( J:194882 )

• 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 but to a much lower extent than on the C57BL/6 background

increased regulatory T cell number ( J:194882 )

• 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

abnormal CD4-positive, alpha-beta T cell physiology ( J:194882 )

• 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

• 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 but to a much lower extent than on the C57BL/6 background

immune system

increased CD4-positive, alpha-beta T cell number ( J:194882 )

• 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 but to a much lower extent than on the C57BL/6 background

increased regulatory T cell number ( J:194882 )

• 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

abnormal CD4-positive, alpha-beta T cell physiology ( J:194882 )

• 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

• 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 but to a much lower extent than on the C57BL/6 background

increased interleukin-13 secretion ( J:194882 )

• 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

respiratory system

respiratory system phenotype ( J:194882 )

N • Background Sensitivity: mice on the BALB/c background do not develop vascular lesions in the lungs or vasoconstriction in pulmonary arteries as seen in mice on a C57BL/6 background