Phenotypes associated with this allele

• Allele Symbol: Ifnar1^tm1Agt
• Allele Name: targeted mutation 1, Michel Aguet
• Allele ID: MGI:1930950
• Summary: 26 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Ifnar1^tm1Agt/Ifnar1^tm1Agt	B6.129S2-Ifnar1^tm1Agt	MGI:3703445
hm2	Ifnar1^tm1Agt/Ifnar1^tm1Agt	involves: 129S2/SvPas	MGI:2654514
hm3	Ifnar1^tm1Agt/Ifnar1^tm1Agt	involves: 129S2/SvPas * 129S6/SvEvTac	MGI:3813912
hm4	Ifnar1^tm1Agt/Ifnar1^tm1Agt	involves: 129S2/SvPas * BALB/c	MGI:6449667
hm5	Ifnar1^tm1Agt/Ifnar1^tm1Agt	involves: 129S2/SvPas * BALB/c * C57BL/6 * SJL	MGI:3765898
hm6	Ifnar1^tm1Agt/Ifnar1^tm1Agt	involves: 129S2/SvPas * C57BL/6	MGI:3526797
cn7	Ifnar1^tm1Agt/Ifnar1^tm1Agt Otulin^tm1c(EUCOMM)Hmgu/Otulin^tm1c(EUCOMM)Hmgu Tg(KRT14-cre)1Cgn/0	involves: 129S2/SvPas * C57BL/6 * C57BL/6N * DBA/2	MGI:7256625
cx8	Ifnar1^tm1Agt/Ifnar1^tm1Agt Myd88^tm1Aki/Myd88^tm1Aki	B6.129-Myd88^tm1Aki Ifnar1^tm1Agt	MGI:5432884
cx9	Ifnar1^tm1Agt/Ifnar1^tm1Agt Myd88^tm1Aki/Myd88^+	B6.129-Myd88^tm1Aki Ifnar1^tm1Agt	MGI:5432885
cx10	Ifnar1^tm1Agt/Ifnar1^tm1Agt Slamf1^tm1Oono/Slamf1^tm1Oono	B6.129-Slamf1^tm1Oono Ifnar1^tm1Agt	MGI:3777089
cx11	Ifnar1^tm1Agt/Ifnar1^tm1Agt Sp140^em1Vnce/Sp140^em1Vnce	B6.Cg-Sp140^em1Vnce Ifnar1^tm1Agt	MGI:7386893
cx12	Ifnar1^tm1Agt/Ifnar1^tm1Agt Tg(H2-K1-Ifnb1)10Seif/Tg(H2-K1-Ifnb1)10Seif	C3.Cg-Ifnar1^tm1Agt Tg(H2-K1-Ifnb1)10Seif	MGI:5141627
cx13	Aim2^tm1.2Arte/Aim2^tm1.2Arte Dnase2a^tm1Osa/Dnase2a^tm1Osa Ifnar1^tm1Agt/Ifnar1^tm1Agt	involves: 129 * BALB/c * C57BL/6 * C57BL/6J	MGI:5823437
cx14	Ifnar1^tm1Agt/Ifnar1^tm1Agt Myd88^tm1Aki/Myd88^tm1Aki	involves: 129P2/OlaHsd * 129S2/SvPas	MGI:4950286
cx15	Dnase2a^tm1Osa/Dnase2a^tm1Osa Ifnar1^tm1Agt/Ifnar1^tm1Agt Xkr8^tm1.2Osa/Xkr8^tm1.2Osa	involves: 129S1/Sv * 129S2/SvPas * 129X1/SvJ * C57BL/6	MGI:7614309
cx16	Dnase2a^tm1Osa/Dnase2a^tm1Osa Ifnar1^tm1Agt/Ifnar1^tm1Agt	involves: 129S1/Sv * 129S2/SvPas * 129X1/SvJ * C57BL/6	MGI:3526798
cx17	Ifnar1^tm1Agt/Ifnar1^tm1Agt Ifngr1^tm1Agt/Ifngr1^tm1Agt	involves: 129S2/SvPas * 129S7/SvEvBrd	MGI:3575517
cx18	Ifnar1^tm1Agt/Ifnar1^tm1Agt Tg(Hmgcr-MX1)LStae/Tg(Hmgcr-MX1)LStae	involves: 129S2/SvPas * BALB/c * C57BL/6 * SJL	MGI:3765897
cx19	Ifnar1^tm1Agt/Ifnar1^tm1Agt Tg(CD46)373Zbz/Tg(CD46)373Zbz	involves: 129S2/SvPas * C3H/He * C57BL/6	MGI:6501995
cx20	Ifnar1^tm1Agt/Ifnar1^tm1Agt Irf2^tm1Mak/Irf2^tm1Mak	involves: 129S2/SvPas * C57BL/6	MGI:3630272
cx21	Ifih1^Rgsc422/Ifih1^+ Ifnar1^tm1Agt/Ifnar1^+	involves: 129S2/SvPas * C57BL/6JJcl * DBA/2JJcl	MGI:5617222
cx22	Ifnar1^tm1Agt/Ifnar1^tm1Agt Rnaseh2b^tm1a(EUCOMM)Wtsi/Rnaseh2b^tm1a(EUCOMM)Wtsi	involves: 129S2/SvPas * C57BL/6N	MGI:5911414
cx23	Adar^em1Stsn/Adar^tm1Olds Ifnar1^tm1Agt/Ifnar1^tm1Agt	involves: 129S/SvEv * 129S2/SvPas * C57BL/6J	MGI:7281848
cx24	Ifnar1^tm1Agt/Ifnar1^tm1Agt Ythdf3^em1Caox/Ythdf3^em1Caox	involves: C57BL/6J	MGI:6479103
cx25	Fas^lpr/Fas^lpr Ifnar1^tm1Agt/Ifnar1^tm1Agt	MRL.Cg-Ifnar1^tm1Agt Fas^lpr	MGI:4838774
cx26	Fas^lpr/Fas^lpr Ifnar1^tm1Agt/Ifnar1^tm1Agt Ifngr1^tm1Agt/Ifngr1^tm1Agt	MRL.Cg-Ifngr1^tm1Agt Ifnar1^tm1Agt Fas^lpr	MGI:4838775

Genotype
MGI:3703445

hm1

• Allelic Composition: Ifnar1^tm1Agt/Ifnar1^tm1Agt
• Genetic Background: B6.129S2-Ifnar1^tm1Agt

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

immune system

abnormal osteoclast morphology ( J:76097 )

• osteoclastogenesis is enhanced

impaired natural killer cell mediated cytotoxicity ( J:118750 )

• natural killer cells from polyI:C-treated mice do not display cytotoxicity towards B16 melanoma cells

decreased interferon-alpha secretion ( J:125611 )

• following infection with Leishmania

decreased interferon-beta secretion ( J:125611 )

• following infection with Leishmania

decreased interleukin-6 secretion ( J:199862 )

• from bone marrow-derived macrophage stimulated with LPS or E. coli K1 E-R8

decreased tumor necrosis factor secretion ( J:199862 )

• from bone marrow-derived macrophage stimulated with LPS or E. coli K1 E-R8

abnormal response to infection ( J:116703 )

• murine cytomegalovirus replication in macrophages is increased greater than 10,000-fold compared to that in wild-type macrophages

increased susceptibility to bacterial infection induced morbidity/mortality ( J:199862 )

• all mice treated with LPS die unlike control mice

neoplasm

increased tumor growth/size ( J:118750 )

• in mice treated with polyI:C the reduction in tumor growth of injected B16 melanoma cells is less than in polyI:C treated control mice

hematopoietic system

abnormal osteoclast morphology ( J:76097 )

• osteoclastogenesis is enhanced

impaired natural killer cell mediated cytotoxicity ( J:118750 )

• natural killer cells from polyI:C-treated mice do not display cytotoxicity towards B16 melanoma cells

skeleton

abnormal osteoclast morphology ( J:76097 )

• osteoclastogenesis is enhanced

decreased bone mineral density ( J:76097 )

mortality/aging

increased susceptibility to bacterial infection induced morbidity/mortality ( J:199862 )

• all mice treated with LPS die unlike control mice

Genotype
MGI:2654514

hm2

• Allelic Composition: Ifnar1^tm1Agt/Ifnar1^tm1Agt
• Genetic Background: involves: 129S2/SvPas

mortality/aging

increased susceptibility to Herpesvirales infection induced morbidity/mortality ( J:115139 )

• MCMV-treated mice exhibit a 800-fold lower LD50 compared with similarly treated wild-type mice

increased susceptibility to Coronaviridae infection induced morbidity/mortality ( J:144400 )

• mice infected with 5 pfu MHV die within 48 hours while wild-type mice survive

increased susceptibility to Paramyxoviridae infection induced morbidity/mortality ( J:160878 )

• 8 of 18 mice inoculated intracerebrally with the attenuated Edmonston measles virus (MV-Edm) die between days 4 and 7 post infection

• 50% of mice inoculated with the rodent brain-adapted neurotrophic measles virus strain CAM/RBH succumb 5-11 days post infection

immune system

immune system phenotype ( J:286237 )

N • mice infected with the Urbani strain of severe acute respiratory syndrome coronavirus (SARS-CoV) or a recombinant isogenic mouse-adapted SARS-CoV (rMA15) that contains six virulence modifying mutations show no increase in susceptibility, pathogenesis, or histological outcomes to infection compared to wild-type controls

abnormal immunoglobulin level ( J:101427 )

• levels of IgG1a, IgG2a and IgG2b neutralizing antibodies are reduced relative to controls after secondary infection with Ectromelia virus

abnormal NK cell physiology ( J:79552 , J:139001 )

• there is 50% less actively proliferating NK cells in the spleen after MCMV infection compared to controls (J:79552)

• NK cells in vivo make much about a fifth less IFN-gamma in response to injections of the TLR9 agonist CpG (J:139001)

impaired natural killer cell mediated cytotoxicity ( J:79552 )

• NK cells from MCMV-infected mice have 10-fold less cytotoxicity than wild-type NK cells

abnormal dendritic cell physiology ( J:114555 )

• in mutant dendritic cells, induction of MHC and costimulatory molecules does not occur in response poly(I:C), in contrast to wild-type cells; LPS or CpG-dependent induction of MHC I and costimulatory molecules is also suppressed

• Ifn beta stimulation of dendritic cells elicits a significantly lower response in mutant cells than in wild-type

• amplification of dendritic signaling is suppressed in mutant dendritic cells stimulated with poly(I:C); RelA activation is suppressed

• induction of MHC and costimulatory molecules is abolished in dendritic cells upon infection with Newcastle disease virus (NDC)

• maturation of dendritic cells is impaired when stimulated by poly(I:C), LPS or NDV, but migration in T cell zone of spleen is not affected

• stimulatory activity of CD4+ and CD8+ T cells by poly(I:C) is impaired in poly(I:C)-stimulated mutant dendritic cells; stimulatory activity of CD8+ T cells is impaired in the LPS- or CpG-stimulated dendritic cells to a lesser extent than in poly(I:C)-stimulated cells

abnormal cytokine secretion ( J:114555 )

• upon viral infection, interferon alpha induction is normal initially, but in later phases does not reach wild-type levels

abnormal inflammatory response ( J:114555 )

• in mutant dendritic cells, induction of MHC and costimulatory molecules does not occur in response poly(I:C), in contrast to wild-type cells; LPS or CpG-dependent induction of MHC I and costimulatory molecules is also suppressed

• MHC I induction is severely impaired in response to all three stimuli

increased susceptibility to Herpesvirales infection ( J:81243 , J:197569 )

• mice are 100-fold more susceptible to murine cytomegalovirus, Smith strain (MCMV) viral infection than controls (J:81243)

• mice infected with herpes simplex type 2 (HSV-2) strain 186 exhibit more severe clinical scores of disease (genital lesions, redness and swelling) compared with wild-type mice (J:197569)

increased susceptibility to Herpesvirales infection induced morbidity/mortality ( J:115139 )

• MCMV-treated mice exhibit a 800-fold lower LD50 compared with similarly treated wild-type mice

increased susceptibility to Poxviridae infection ( J:101427 )

• survive secondary infections

• increased susceptibility to Ectromelia virus

• 100% mortality by day 6-12 after primary infection infection

• elevated virus titers in all organs tested after primary infection but much improved after secondary infections

increased susceptibility to Coronaviridae infection ( J:144400 )

• mice show increased susceptibility to infection with murine hepatitis virus (MHV) A59 (a coronavirus) , showing increased viral titers and developing a rapidly lethal acute hemorrhagic liver disease

• plasmacytoid dendritic cells (pDCs) are more susceptible to MHV infection than wild-type pDCs

increased susceptibility to Coronaviridae infection induced morbidity/mortality ( J:144400 )

• mice infected with 5 pfu MHV die within 48 hours while wild-type mice survive

increased susceptibility to Paramyxoviridae infection ( J:160878 )

• mice inoculated intranasally with the attenuated Edmonston measles virus (MV-Edm) show macroscopic purple lesion areas in the lungs

• 8 of 18 mice inoculated intracerebrally with MV-Edm die between days 4 and 7 post infection and show signs of neurological disease including initial hyperactivity followed by awkward gait, lethargy, lack of mobility, and death

• susceptibility to MV-Edm infection is more pronounced if less virus is inoculated

increased susceptibility to Paramyxoviridae infection induced morbidity/mortality ( J:160878 )

• 8 of 18 mice inoculated intracerebrally with the attenuated Edmonston measles virus (MV-Edm) die between days 4 and 7 post infection

• 50% of mice inoculated with the rodent brain-adapted neurotrophic measles virus strain CAM/RBH succumb 5-11 days post infection

increased susceptibility to Picornaviridae infection ( J:137522 )

• increased susceptibility of encephalomyocarditis virus induced lethality

liver/biliary system

liver hemorrhage ( J:144400 )

• MHV-infected mice develop an acute hemorrhagic liver disease with massive hepatocyte necrosis

vision/eye

cornea vascularization ( J:114973 )

• corneal injection of a plasmid encoding short hairpin RNA targeting the C terminus of the secreted form of Flt1 (pshRNA-sflt1) induces corneal vascularization within 3 days of injection in wild-type and mutant mice

cardiovascular system

liver hemorrhage ( J:144400 )

• MHV-infected mice develop an acute hemorrhagic liver disease with massive hepatocyte necrosis

cornea vascularization ( J:114973 )

• corneal injection of a plasmid encoding short hairpin RNA targeting the C terminus of the secreted form of Flt1 (pshRNA-sflt1) induces corneal vascularization within 3 days of injection in wild-type and mutant mice

hematopoietic system

abnormal immunoglobulin level ( J:101427 )

• levels of IgG1a, IgG2a and IgG2b neutralizing antibodies are reduced relative to controls after secondary infection with Ectromelia virus

abnormal NK cell physiology ( J:79552 , J:139001 )

• there is 50% less actively proliferating NK cells in the spleen after MCMV infection compared to controls (J:79552)

• NK cells in vivo make much about a fifth less IFN-gamma in response to injections of the TLR9 agonist CpG (J:139001)

impaired natural killer cell mediated cytotoxicity ( J:79552 )

• NK cells from MCMV-infected mice have 10-fold less cytotoxicity than wild-type NK cells

homeostasis/metabolism

increased circulating alanine transaminase level ( J:144400 )

• MHV-infected mice show rapidly rising liver enzyme values in serum

behavior/neurological

lethargy ( J:160878 )

• mice inoculated intracerebrally with MV-Edm show initial hyperactivity that is followed by awkward gait and lethargy

abnormal gait ( J:160878 )

• mice inoculated intracerebrally with MV-Edm show initial hyperactivity followed by awkward gait and lack of mobility

induced hyperactivity ( J:160878 )

• mice inoculated intracerebrally with MV-Edm show initial hyperactivity following infection

Genotype
MGI:3813912

hm3

• Allelic Composition: Ifnar1^tm1Agt/Ifnar1^tm1Agt
• Genetic Background: involves: 129S2/SvPas * 129S6/SvEvTac

immune system

abnormal CD8-positive, alpha-beta T cell physiology ( J:54482 )

• the antigen specificity of CD8 T cells to common LCMV-derived peptides differs from controls with a higher percentage being specific for the GP33-41 peptide

increased circulating interleukin-12 level ( J:54482 )

• 3-4 pg/ml of IL-12 heterodimer are present in the serum 1.5- and 3- days after infection with LCMV while no IL-12 is detected at this timepoint in wild-type mice

abnormal interferon-gamma secretion ( J:54482 )

• splenic leukocyte production of IFN-gamma is increased 3-fold 8 days after infection with LCMV compared to controls

• two-thirds of this IFN-gamma production is dependent on CD8 T cells

• these T cells produce less IFN-gamma on a per cell basis

increased susceptibility to Riboviria infection ( J:54482 )

• mice have a 2-log higher viral titer in the spleen, and a 4-log higher viral titer in the liver, 4.5 days after infection with LCMV virus

• mice took much longer to clear virus, around 28 days, compared to 7 days for wild-type mice

homeostasis/metabolism

increased circulating interleukin-12 level ( J:54482 )

• 3-4 pg/ml of IL-12 heterodimer are present in the serum 1.5- and 3- days after infection with LCMV while no IL-12 is detected at this timepoint in wild-type mice

hematopoietic system

abnormal CD8-positive, alpha-beta T cell physiology ( J:54482 )

• the antigen specificity of CD8 T cells to common LCMV-derived peptides differs from controls with a higher percentage being specific for the GP33-41 peptide

Genotype
MGI:6449667

hm4

• Allelic Composition: Ifnar1^tm1Agt/Ifnar1^tm1Agt
• Genetic Background: involves: 129S2/SvPas * BALB/c

mortality/aging

increased susceptibility to Coronaviridae infection induced morbidity/mortality ( J:291874 )

• all mice infected with murine hepatitis virus strain 1 (MHV-1) succumb to a sub-lethal dose compared to only about 10% of control BALB/c mice

increased susceptibility to Orthomyxoviridae infection induced morbidity/mortality ( J:291874 )

• mice infected with the mouse-adapted H1N1 influenza A virus, PR8 strain, succumb to a sub-lethal dose compared to only about 20% of control BALB/c mice

immune system

decreased T cell apoptosis ( J:291874 )

• SARS-CoV-MA15-infected mice show lower levels of CD8 and CD4 T cell apoptos

increased CD4-positive, alpha-beta T cell number ( J:291874 )

• SARS-CoV-MA15-infected mice show increased numbers of virus-specific CD4 T cells in lungs

increased CD8-positive, alpha-beta T cell number ( J:291874 )

• SARS-CoV-MA15-infected mice show increased numbers of virus-specific CD8 T cells in lungs

abnormal mononuclear phagocyte morphology ( J:291874 )

• SARS-CoV-MA15-infected mice do show an increase in highly activated inflammatory monocyte-macrophages as is seen in infected BALB/c controls

• SARS-CoV-MA15-infected mice show a decrease in the percentage and numbers of inflammatory monocyte-macrophages that produce the pro-inflammatory cytokines TNF, IL-6, IL1-beta, and iNOS

decreased Ly6C high monocyte number ( J:291874 )

• SARS-CoV-MA15-infected mice do not show an increase in Ly6C^hiCD11b⁺ cells in the lungs as is seen in controls

abnormal cytokine level ( J:291874 )

• SARS-CoV-MA15- infected mice have reduced levels of cytokines and chemokines in the broncho-alveolar lavage fluid

increased susceptibility to Coronaviridae infection induced morbidity/mortality ( J:291874 )

• all mice infected with murine hepatitis virus strain 1 (MHV-1) succumb to a sub-lethal dose compared to only about 10% of control BALB/c mice

increased susceptibility to Orthomyxoviridae infection induced morbidity/mortality ( J:291874 )

• mice infected with the mouse-adapted H1N1 influenza A virus, PR8 strain, succumb to a sub-lethal dose compared to only about 20% of control BALB/c mice

decreased susceptibility to Coronaviridae infection ( J:291874 )

• mice infected with a lethal dose of the mouse adapted severe acute respiratory syndrome coronavirus (SARS-CoV-MA15) survive the infection, exhibiting only a moderate 15% weight loss and mild to moderate clinical disease

• middle-aged (8-9 month old) mice show increased survival after SARS-CoV-MA15 infection compared to middle-aged BALB/c control mice

• total lung virus loads after SARS-CoV-MA15 infection are the same as in control mice, except for a modest increase at 3 days post infection (dpi) in mutants

• SARS-CoV-MA15 virus is completely cleared from the lungs by 10 dpi

• SARS-CoV-MA15-infected mice show nearly normal lungs at 3 and 5 dpi compared to hyperemia and congestion in controls, with minimal alveolar edema and increased peribronchial-perivascular immune cell infiltration, and reduced lung microvascular leakage

homeostasis/metabolism

abnormal cytokine level ( J:291874 )

• SARS-CoV-MA15- infected mice have reduced levels of cytokines and chemokines in the broncho-alveolar lavage fluid

hematopoietic system

decreased T cell apoptosis ( J:291874 )

• SARS-CoV-MA15-infected mice show lower levels of CD8 and CD4 T cell apoptos

increased CD4-positive, alpha-beta T cell number ( J:291874 )

• SARS-CoV-MA15-infected mice show increased numbers of virus-specific CD4 T cells in lungs

increased CD8-positive, alpha-beta T cell number ( J:291874 )

• SARS-CoV-MA15-infected mice show increased numbers of virus-specific CD8 T cells in lungs

abnormal mononuclear phagocyte morphology ( J:291874 )

• SARS-CoV-MA15-infected mice do show an increase in highly activated inflammatory monocyte-macrophages as is seen in infected BALB/c controls

• SARS-CoV-MA15-infected mice show a decrease in the percentage and numbers of inflammatory monocyte-macrophages that produce the pro-inflammatory cytokines TNF, IL-6, IL1-beta, and iNOS

decreased Ly6C high monocyte number ( J:291874 )

• SARS-CoV-MA15-infected mice do not show an increase in Ly6C^hiCD11b⁺ cells in the lungs as is seen in controls

cellular

decreased T cell apoptosis ( J:291874 )

• SARS-CoV-MA15-infected mice show lower levels of CD8 and CD4 T cell apoptos

Genotype
MGI:3765898

hm5

• Allelic Composition: Ifnar1^tm1Agt/Ifnar1^tm1Agt
• Genetic Background: involves: 129S2/SvPas * BALB/c * C57BL/6 * SJL

immune system

increased susceptibility to Orthomyxoviridae infection ( J:119783 )

• mice infected with Thogoto virus all succumb within 3 days compared to 5 days for littermate controls

Genotype
MGI:3526797

hm6

• Allelic Composition: Ifnar1^tm1Agt/Ifnar1^tm1Agt
• Genetic Background: involves: 129S2/SvPas * C57BL/6

immune system

immune system phenotype ( J:238323 )

N • mice do not develop chronic polyarthritis

abnormal susceptibility to infection ( J:120938 )

• infection with hvPR8 produces high titers of 5x10⁷

• infection with lvPR8 produces low titers of 5x10⁴

Genotype
MGI:7256625

cn7

• Allelic Composition: Ifnar1^tm1Agt/Ifnar1^tm1Agt; Otulin^{tm1c(EUCOMM)Hmgu}/Otulin^{tm1c(EUCOMM)Hmgu}; Tg(KRT14-cre)1Cgn/0
• Genetic Background: involves: 129S2/SvPas * C57BL/6 * C57BL/6N * DBA/2

immune system

immune system phenotype ( J:312394 )

N • normal circulating inflammatory cytokine and chemokine levels in most mice

dermatitis ( J:312394 )

• delineated inflamed skin lesions from age 6 weeks in some mice

integument

integument phenotype ( J:312394 )

N • no skin lesions and normal epidermal thickness in most mice

dermatitis ( J:312394 )

• delineated inflamed skin lesions from age 6 weeks in some mice

Genotype
MGI:5432884

cx8

• Allelic Composition: Ifnar1^tm1Agt/Ifnar1^tm1Agt; Myd88^tm1Aki/Myd88^tm1Aki
• Genetic Background: B6.129-Myd88^tm1Aki Ifnar1^tm1Agt

mortality/aging

increased susceptibility to parasitic infection induced morbidity/mortality ( J:140485 )

• all mice die within 19 days of infection with T. cruzi

immune system

increased susceptibility to parasitic infection ( J:140485 )

• in cultured bone marrow dendritic cells from mice infected with Trypanosoma cruzi the number of trypomastigotes released into the supernatant is considerably increased and the replication within macrophages is markedly enhanced compared to controls

• serum parasite counts continue to increase after day 13 and reach a much higher level by 15 days post infection

increased susceptibility to parasitic infection induced morbidity/mortality ( J:140485 )

• all mice die within 19 days of infection with T. cruzi

Genotype
MGI:5432885

cx9

• Allelic Composition: Ifnar1^tm1Agt/Ifnar1^tm1Agt; Myd88^tm1Aki/Myd88⁺
• Genetic Background: B6.129-Myd88^tm1Aki Ifnar1^tm1Agt

immune system

increased susceptibility to parasitic infection ( J:140485 )

• in cultured bone marrow dendritic cells from mice infected with Trypanosoma cruzi the number of trypomastigotes released into the supernatant is slightly increased and the replication within macrophages is slightly enhanced compared to controls

• slight increase in the number of trypomastigotes in the serum of T. cruzi infected mice

Genotype
MGI:3777089

cx10

• Allelic Composition: Ifnar1^tm1Agt/Ifnar1^tm1Agt; Slamf1^tm1Oono/Slamf1^tm1Oono
• Genetic Background: B6.129-Slamf1^tm1Oono Ifnar1^tm1Agt

immune system

abnormal lymphopoiesis ( J:117810 )

• lymphocytes from measle virus infected double mutant show proliferation inhibition when stimulated with ConA

increased susceptibility to Paramyxoviridae infection ( J:117810 )

• double mutant mice permitted the in vivo growth of measle virus after i.p. or intranasal inoculation, however, on the contrary to expectation, growth of IC-B strain measle virus was not enhanced in splenocytes form double mutant compared with those from the single mutant, Ifnar1⁺/Ifnar1⁺, Slamf1^tm1Oono/Slamf1^tm1Oono, splenocytes

hematopoietic system

abnormal lymphopoiesis ( J:117810 )

• lymphocytes from measle virus infected double mutant show proliferation inhibition when stimulated with ConA

Genotype
MGI:7386893

cx11

• Allelic Composition: Ifnar1^tm1Agt/Ifnar1^tm1Agt; Sp140^em1Vnce/Sp140^em1Vnce
• Genetic Background: B6.Cg-Sp140^em1Vnce Ifnar1^tm1Agt

immune system

immune system phenotype ( J:313901 )

N • mice exhibit normal susceptibility to M. tuberculosis infection

Genotype
MGI:5141627

cx12

• Allelic Composition: Ifnar1^tm1Agt/Ifnar1^tm1Agt; Tg(H2-K1-Ifnb1)10Seif/Tg(H2-K1-Ifnb1)10Seif
• Genetic Background: C3.Cg-Ifnar1^tm1Agt Tg(H2-K1-Ifnb1)10Seif

reproductive system

reproductive system phenotype ( J:174828 )

N • mice exhibit normal fertility

N • male mice exhibit normal sperm parameters

Genotype
MGI:5823437

cx13

• Allelic Composition: Aim2^tm1.2Arte/Aim2^tm1.2Arte; Dnase2a^tm1Osa/Dnase2a^tm1Osa; Ifnar1^tm1Agt/Ifnar1^tm1Agt
• Genetic Background: involves: 129 * BALB/c * C57BL/6 * C57BL/6J

hematopoietic system

abnormal spleen morphology ( J:238323 )

• increase in expression of IFN-beta in splenic tissue

enlarged spleen ( J:238323 )

• splenomegaly, with spleen sizes being 4-5 fold increased

increased spleen weight ( J:238323 )

homeostasis/metabolism

increased circulating interleukin-10 level ( J:238323 )

increased circulating tumor necrosis factor level ( J:238323 )

immune system

abnormal spleen morphology ( J:238323 )

• increase in expression of IFN-beta in splenic tissue

enlarged spleen ( J:238323 )

• splenomegaly, with spleen sizes being 4-5 fold increased

increased spleen weight ( J:238323 )

increased circulating interleukin-10 level ( J:238323 )

increased circulating tumor necrosis factor level ( J:238323 )

increased autoantibody level ( J:238323 )

• presence of anti-cyclic citrullinated peptide antibodies

rheumatoid arthritis ( J:238323 )

• signs of polyarthritis are reduced compared to double Dnase2a and Ifnar1 homozygotes, although mice do show some arthritis development

• joints do not show an increase in cleaved caspase-1

• expression of pro-inflammatory cytokines, such as TNF, IL-6, and IL-1beta, is reduced both at protein and mRNA level in joints compared to double Dnase2a and Ifnar1 homozygotes

• mice show decreased macrophage infiltration in joints compared to double Dnase2a and Ifnar1 homozygotes

skeleton

rheumatoid arthritis ( J:238323 )

• signs of polyarthritis are reduced compared to double Dnase2a and Ifnar1 homozygotes, although mice do show some arthritis development

• joints do not show an increase in cleaved caspase-1

• expression of pro-inflammatory cytokines, such as TNF, IL-6, and IL-1beta, is reduced both at protein and mRNA level in joints compared to double Dnase2a and Ifnar1 homozygotes

• mice show decreased macrophage infiltration in joints compared to double Dnase2a and Ifnar1 homozygotes

growth/size/body

enlarged spleen ( J:238323 )

• splenomegaly, with spleen sizes being 4-5 fold increased

increased spleen weight ( J:238323 )

Genotype
MGI:4950286

cx14

• Allelic Composition: Ifnar1^tm1Agt/Ifnar1^tm1Agt; Myd88^tm1Aki/Myd88^tm1Aki
• Genetic Background: involves: 129P2/OlaHsd * 129S2/SvPas

immune system

abnormal leukocyte migration ( J:171379 )

• LPS-induced monocyte emigration is decreased compared to in similarly treated wild-type mice

cellular

abnormal leukocyte migration ( J:171379 )

• LPS-induced monocyte emigration is decreased compared to in similarly treated wild-type mice

hematopoietic system

abnormal leukocyte migration ( J:171379 )

• LPS-induced monocyte emigration is decreased compared to in similarly treated wild-type mice

Genotype
MGI:7614309

cx15

• Allelic Composition: Dnase2a^tm1Osa/Dnase2a^tm1Osa; Ifnar1^tm1Agt/Ifnar1^tm1Agt; Xkr8^tm1.2Osa/Xkr8^tm1.2Osa
• Genetic Background: involves: 129S1/Sv * 129S2/SvPas * 129X1/SvJ * C57BL/6

reproductive system

abnormal Sertoli cell phagocytosis ( J:293092 )

• at 3 weeks of age, apoptotic germ cells are not engulfed by Sertoli cells; the number of Sertoli cells carrying undigested DNA (foci) is significantly lower than that in testes that are heterozygous for Xkr8^tm1.2Osa and homozygous for both Dnase2a^tm1Osa and Ifnar1^tm1Agt

• although abnormal foci are less abundant, many clusters of hematoxylin-positive materials are found in peripheral regions of the testicular tubes, suggesting that these represent unengulfed apoptotic bodies with condensed and fragmented nuclei

Genotype
MGI:3526798

cx16

• Allelic Composition: Dnase2a^tm1Osa/Dnase2a^tm1Osa; Ifnar1^tm1Agt/Ifnar1^tm1Agt
• Genetic Background: involves: 129S1/Sv * 129S2/SvPas * 129X1/SvJ * C57BL/6

growth/size/body

postnatal growth retardation ( J:114982 )

enlarged spleen ( J:114982 , J:238323 )

• develop splenomegaly from 1 month of age, which becomes prominent as mutants age (J:114982)

• splenomegaly, with spleen sizes being 4-5 fold increased (J:238323)

increased spleen weight ( J:238323 )

immune system

abnormal macrophage morphology ( J:114982 )

• exhibit numerous abnormal macrophages that contain DNA in lysosomes in the bone marrow, spleen and other tissues

abnormal spleen morphology ( J:238323 )

• increase in expression of IFN-beta in splenic tissue

enlarged spleen ( J:114982 , J:238323 )

• develop splenomegaly from 1 month of age, which becomes prominent as mutants age (J:114982)

• splenomegaly, with spleen sizes being 4-5 fold increased (J:238323)

increased spleen weight ( J:238323 )

increased spleen red pulp amount ( J:114982 )

• splenomegaly due to enlargement of the red pulp

abnormal cytokine level ( J:114982 )

• affected joints show 5-100 fold elevation of TNF-alpha, IL-1beta, IL-6, IL-10, IFN-beta, and IFN-gamma mRNA levels, the set of cytokines that are elevated in joints of humans with rheumatoid arthritis

increased circulating interleukin-10 level ( J:238323 )

increased circulating interleukin-18 level ( J:114982 )

• serum shows elevated levels of IL-18 protein

increased circulating tumor necrosis factor level ( J:114982 , J:238323 )

• serum shows elevated levels of TNF-alpha at 4-6 weeks of age, before joints show abnormalities; the extend of elevation decreases as mutants age (J:114982)

increased autoantibody level ( J:238323 )

• presence of anti-cyclic citrullinated peptide antibodies

increased anti-double stranded DNA antibody level ( J:114982 )

• level of anti-double-stranded DNA antibody is elevated 3-fold

joint inflammation ( J:114982 , J:238323 )

• joints of show severe synovitis with villus proliferation accompanied by pannus formation; pannus fills the joint cavity, erodes cartilage, destroys bones, and occasionally penetrates the bone marrow (J:114982)

• exhibit infiltration of subsynovial tissues by T cells and neutrophils (J:114982)

• joints show severe signs of synovitis with hyperproliferation of synovial cells and massive immune cell infiltration at 15 months of age, associated with panus formation, cartilage destruction, and bone erosion (J:238323)

rheumatoid arthritis ( J:114982 , J:238323 )

• develop polyarthritis as age; forelimbs and hindlimbs begin to swell at 2-3 months of age and swelling first affects the digits, then the foot and finally the wrists and ankles (J:114982)

• mice develop chronic polyarthritis that progresses with age (J:238323)

• joints show an increase in cleaved caspase-1, indicating signs of inflammasome activation (J:238323)

• expression of pro-inflammatory cytokines such as TNF, IL-6, and IL-1beta is increased both at protein and mRNA level in joints (J:238323)

• immune cell infiltrate is mainly dominated by presence of macrophages (J:238323)

hematopoietic system

anemia ( J:114982 )

• slightly anemic

abnormal macrophage morphology ( J:114982 )

• exhibit numerous abnormal macrophages that contain DNA in lysosomes in the bone marrow, spleen and other tissues

abnormal spleen morphology ( J:238323 )

• increase in expression of IFN-beta in splenic tissue

enlarged spleen ( J:114982 , J:238323 )

• develop splenomegaly from 1 month of age, which becomes prominent as mutants age (J:114982)

• splenomegaly, with spleen sizes being 4-5 fold increased (J:238323)

increased spleen weight ( J:238323 )

increased spleen red pulp amount ( J:114982 )

• splenomegaly due to enlargement of the red pulp

homeostasis/metabolism

abnormal blood homeostasis ( J:114982 )

• carry a significant level of DNA in serum which decreases as mutants age

abnormal cytokine level ( J:114982 )

• affected joints show 5-100 fold elevation of TNF-alpha, IL-1beta, IL-6, IL-10, IFN-beta, and IFN-gamma mRNA levels, the set of cytokines that are elevated in joints of humans with rheumatoid arthritis

increased circulating interleukin-10 level ( J:238323 )

increased circulating interleukin-18 level ( J:114982 )

• serum shows elevated levels of IL-18 protein

increased circulating tumor necrosis factor level ( J:114982 , J:238323 )

• serum shows elevated levels of TNF-alpha at 4-6 weeks of age, before joints show abnormalities; the extend of elevation decreases as mutants age (J:114982)

skeleton

joint inflammation ( J:114982 , J:238323 )

• joints of show severe synovitis with villus proliferation accompanied by pannus formation; pannus fills the joint cavity, erodes cartilage, destroys bones, and occasionally penetrates the bone marrow (J:114982)

• exhibit infiltration of subsynovial tissues by T cells and neutrophils (J:114982)

• joints show severe signs of synovitis with hyperproliferation of synovial cells and massive immune cell infiltration at 15 months of age, associated with panus formation, cartilage destruction, and bone erosion (J:238323)

rheumatoid arthritis ( J:114982 , J:238323 )

• develop polyarthritis as age; forelimbs and hindlimbs begin to swell at 2-3 months of age and swelling first affects the digits, then the foot and finally the wrists and ankles (J:114982)

• mice develop chronic polyarthritis that progresses with age (J:238323)

• joints show an increase in cleaved caspase-1, indicating signs of inflammasome activation (J:238323)

• expression of pro-inflammatory cytokines such as TNF, IL-6, and IL-1beta is increased both at protein and mRNA level in joints (J:238323)

• immune cell infiltrate is mainly dominated by presence of macrophages (J:238323)

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
rheumatoid arthritis		DOID:7148	OMIM:180300	J:114982 , J:238323

Genotype
MGI:3575517

cx17

• Allelic Composition: Ifnar1^tm1Agt/Ifnar1^tm1Agt; Ifngr1^tm1Agt/Ifngr1^tm1Agt
• Genetic Background: involves: 129S2/SvPas * 129S7/SvEvBrd

mortality/aging

increased susceptibility to Herpesvirales infection induced morbidity/mortality ( J:115139 )

• all mice are killed at as little as 10 PFU of MCMV

immune system

immune system phenotype ( J:286237 )

N • mice infected with the Urbani strain of severe acute respiratory syndrome coronavirus (SARS-CoV) show no differences in susceptibility to infection from wild-type or single homozygous mice

abnormal immunoglobulin level ( J:101427 )

• levels of IgG2a and IgG2b neutralizing antibodies are reduced relative to controls after secondary infection with Ectromelia virus

increased susceptibility to Herpesvirales infection ( J:81243 )

• mice are 100,000-fold more susceptible to MCMV viral infection than controls

increased susceptibility to Herpesvirales infection induced morbidity/mortality ( J:115139 )

• all mice are killed at as little as 10 PFU of MCMV

increased susceptibility to Poxviridae infection ( J:101427 )

• increased susceptibility to Ectromelia virus

• 100% mortality by day 6-12 after primary infection infection

• elevated virus titers in all organs tested after primary infection but much improved after secondary infections

• survive secondary infections

increased susceptibility to Togaviridae infection ( J:81243 )

• acutely sensitive to Sindbis viral infection; as little as one pfu will induce mortality as opposed to wild-type, which can withstand doses of 100,000 pfu

hematopoietic system

abnormal immunoglobulin level ( J:101427 )

• levels of IgG2a and IgG2b neutralizing antibodies are reduced relative to controls after secondary infection with Ectromelia virus

Genotype
MGI:3765897

cx18

• Allelic Composition: Ifnar1^tm1Agt/Ifnar1^tm1Agt; Tg(Hmgcr-MX1)LStae/Tg(Hmgcr-MX1)LStae
• Genetic Background: involves: 129S2/SvPas * BALB/c * C57BL/6 * SJL

mortality/aging

decreased susceptibility to Orthomyxoviridae infection induced morbidity/mortality ( J:119783 )

• mice infected with Thogoto virus all survive while littermate controls die within 5 days

• only 43% of mice die from La Crosse virus infection as opposed to Ifnar1^tm1Agt mice in which 94% die

• only 59% of mice die from Semliki Forest virus infection as opposed to 100% of Ifnar1^tm1Agt mice

immune system

decreased susceptibility to Orthomyxoviridae infection ( J:119783 )

• no Thogoto virus is detectable in liver 36 hours after infection

decreased susceptibility to Orthomyxoviridae infection induced morbidity/mortality ( J:119783 )

• mice infected with Thogoto virus all survive while littermate controls die within 5 days

• only 43% of mice die from La Crosse virus infection as opposed to Ifnar1^tm1Agt mice in which 94% die

• only 59% of mice die from Semliki Forest virus infection as opposed to 100% of Ifnar1^tm1Agt mice

Genotype
MGI:6501995

cx19

• Allelic Composition: Ifnar1^tm1Agt/Ifnar1^tm1Agt; Tg(CD46)373Zbz/Tg(CD46)373Zbz
• Genetic Background: involves: 129S2/SvPas * C3H/He * C57BL/6

mortality/aging

increased susceptibility to Paramyxoviridae infection induced morbidity/mortality ( J:160878 )

• mice inoculated intracerebrally with the attenuated Edmonston measles virus (MV-Edm) show increased lethality after two days post infection

• 50% of mice inoculated with the rat brain-adapted neurotrophic measles virus strain CAM/RBH succumb 5-11 days post infection

immune system

brain inflammation ( J:160878 )

• mice infected intracerebrally with MV-Edm show encephalitis

meningitis ( J:160878 )

• mice infected intracerebrally with MV-Edm exhibit meningitis with inflammatory infiltrates of leukocytes and extensive vacuolization and necrosis of nearby brain parenchyma

lung inflammation ( J:160878 )

• mice inoculated intranasally with MV-Edm show acute lung inflammation

increased susceptibility to Paramyxoviridae infection ( J:160878 )

• mice inoculated intranasally with the attenuated Edmonston measles virus (MV-Edm) show larger purple lesions in the lungs than seen in Ifnar1^tm1Agt homozygotes

• mice inoculated intranasally with MV-Edm show acute lung inflammation, extensive hyperemia, and diffuse hemorrhage in large areas of the lungs and show peak virus levels in the lungs at day 6 post infection which spreads to liver, spleen, kidney, and peripheral blood mononuclear cells

• susceptibility to MV-Edm infection is more pronounced if less virus is inoculated

• mice inoculated intracerebrally with MV-Edm show increased lethality after two days post infection and show clinical signs of neural disease, including initial hyperactivity followed by awkward gait, lethargy, lack of mobility, and death

increased susceptibility to Paramyxoviridae infection induced morbidity/mortality ( J:160878 )

• mice inoculated intracerebrally with the attenuated Edmonston measles virus (MV-Edm) show increased lethality after two days post infection

• 50% of mice inoculated with the rat brain-adapted neurotrophic measles virus strain CAM/RBH succumb 5-11 days post infection

respiratory system

lung hemorrhage ( J:160878 )

• mice inoculated intranasally with MV-Edm show diffuse hemorrhage in large areas of the lungs

pulmonary hyperemia ( J:160878 )

• mice inoculated intranasally with MV-Edm show extensive hyperemia

lung inflammation ( J:160878 )

• mice inoculated intranasally with MV-Edm show acute lung inflammation

nervous system

brain inflammation ( J:160878 )

• mice infected intracerebrally with MV-Edm show encephalitis

meningitis ( J:160878 )

• mice infected intracerebrally with MV-Edm exhibit meningitis with inflammatory infiltrates of leukocytes and extensive vacuolization and necrosis of nearby brain parenchyma

astrocytosis ( J:160878 )

• mice infected intracerebrally with MV-Edm show numerous reactive astrocytes in many brain areas

abnormal neuron physiology ( J:160878 )

• mice infected intracerebrally with MV-Edm exhibit necrosis of neurons in the cerebral cortex, corpus callosum, and hypothalamus

behavior/neurological

lethargy ( J:160878 )

• mice inoculated intracerebrally with MV-Edm show initial hyperactivity that is followed by awkward gait and lethargy

abnormal gait ( J:160878 )

• mice inoculated intracerebrally with MV-Edm show initial hyperactivity followed by awkward gait and lack of mobility

induced hyperactivity ( J:160878 )

• mice inoculated intracerebrally with MV-Edm show initial hyperactivity following infection

cardiovascular system

lung hemorrhage ( J:160878 )

• mice inoculated intranasally with MV-Edm show diffuse hemorrhage in large areas of the lungs

pulmonary hyperemia ( J:160878 )

• mice inoculated intranasally with MV-Edm show extensive hyperemia

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
measles		DOID:8622		J:160878

Genotype
MGI:3630272

cx20

• Allelic Composition: Ifnar1^tm1Agt/Ifnar1^tm1Agt; Irf2^tm1Mak/Irf2^tm1Mak
• Genetic Background: involves: 129S2/SvPas * C57BL/6

integument

abnormal skin condition ( J:66034 )

• no skin symptoms are observed up to 4 months of age compared to Irf2-null mice

Genotype
MGI:5617222

cx21

• Allelic Composition: Ifih1^Rgsc422/Ifih1⁺; Ifnar1^tm1Agt/Ifnar1⁺
• Genetic Background: involves: 129S2/SvPas * C57BL/6JJcl * DBA/2JJcl

immune system

glomerulonephritis ( J:209917 )

• less severe than in mice wild-type for Ifnar1

renal/urinary system

glomerulonephritis ( J:209917 )

• less severe than in mice wild-type for Ifnar1

Genotype
MGI:5911414

cx22

• Allelic Composition: Ifnar1^tm1Agt/Ifnar1^tm1Agt; Rnaseh2b^{tm1a(EUCOMM)Wtsi}/Rnaseh2b^{tm1a(EUCOMM)Wtsi}
• Genetic Background: involves: 129S2/SvPas * C57BL/6N
• Cell Lines: EPD0087_4_A02

mortality/aging

perinatal lethality, complete penetrance ( J:186986 )

growth/size/body

decreased fetal size ( J:186986 )

• at E17.5

Genotype
MGI:7281848

cx23

• Allelic Composition: Adar^em1Stsn/Adar^tm1Olds; Ifnar1^tm1Agt/Ifnar1^tm1Agt
• Genetic Background: involves: 129S/SvEv * 129S2/SvPas * C57BL/6J

growth/size/body

growth/size/body region phenotype ( J:308678 )

• normal weight, weight gain and growth

homeostasis/metabolism

homeostasis/metabolism phenotype ( J:308678 )

N • normal expression of interferon-stimulated genes in cerebellum, kidney and liver

N • normal expression of integrated stress response (ISR) in cerebellum, kidney and liver

mortality/aging

mortality/aging ( J:308678 )

N • normal survival

Genotype
MGI:6479103

cx24

• Allelic Composition: Ifnar1^tm1Agt/Ifnar1^tm1Agt; Ythdf3^em1Caox/Ythdf3^em1Caox
• Genetic Background: involves: C57BL/6J

immune system

abnormal macrophage physiology ( J:276397 )

• following infection with vesicular stomatitis virus (VSV) , peritoneal macrophages from double mutant mice show abrogation of both the IFN-stimulated gene activation and enhanced antiviral response observed in single Ythdf3^em1Caox homozygotes

hematopoietic system

abnormal macrophage physiology ( J:276397 )

• following infection with vesicular stomatitis virus (VSV) , peritoneal macrophages from double mutant mice show abrogation of both the IFN-stimulated gene activation and enhanced antiviral response observed in single Ythdf3^em1Caox homozygotes

Genotype
MGI:4838774

cx25

• Allelic Composition: Fas^lpr/Fas^lpr; Ifnar1^tm1Agt/Ifnar1^tm1Agt
• Genetic Background: MRL.Cg-Ifnar1^tm1Agt Fas^lpr

immune system

salivary gland inflammation ( J:92084 )

• worse than in Fas^lpr homozygotes

increased IgA level ( J:92084 )

• compared with Fas^lpr homozygotes

increased IgG level ( J:92084 )

• mice exhibit IgG deposits in the kidney unlike wild-type mice

increased IgG2b level ( J:92084 )

• compared with Fas^lpr homozygotes

increased IgG3 level ( J:92084 )

• compared with Fas^lpr homozygotes

increased IgM level ( J:92084 )

• compared with Fas^lpr homozygotes

enlarged lymph nodes ( J:92084 )

increased susceptibility to systemic lupus erythematosus ( J:92084 )

• mice exhibit increased serum levels of kappa-chain rheumatoid factor and DNA auto-antibodies, lymphadenopathy, glomerulonephritis, renal immunoglobulin deposits, proteinuria, and end organ disease compared to in wild-type mice

• some systemic lupus erythematosus symptoms are more severe than Fas^lpr homozygotes

increased autoantibody level ( J:92084 )

• mice exhibit increased serum levels of kappa-chain rheumatoid factor compared to in wild-type mice and Fas^lpr homozygotes

increased anti-nuclear antigen antibody level ( J:92084 )

• DNA auto-antibodies are increased compared to in wild-type mice at 12 weeks

glomerulonephritis ( J:92084 )

renal/urinary system

increased urine protein level ( J:92084 )

glomerulonephritis ( J:92084 )

homeostasis/metabolism

increased urine protein level ( J:92084 )

endocrine/exocrine glands

salivary gland inflammation ( J:92084 )

• worse than in Fas^lpr homozygotes

digestive/alimentary system

salivary gland inflammation ( J:92084 )

• worse than in Fas^lpr homozygotes

hematopoietic system

increased IgA level ( J:92084 )

• compared with Fas^lpr homozygotes

increased IgG level ( J:92084 )

• mice exhibit IgG deposits in the kidney unlike wild-type mice

increased IgG2b level ( J:92084 )

• compared with Fas^lpr homozygotes

increased IgG3 level ( J:92084 )

• compared with Fas^lpr homozygotes

increased IgM level ( J:92084 )

• compared with Fas^lpr homozygotes

Genotype
MGI:4838775

cx26

• Allelic Composition: Fas^lpr/Fas^lpr; Ifnar1^tm1Agt/Ifnar1^tm1Agt; Ifngr1^tm1Agt/Ifngr1^tm1Agt
• Genetic Background: MRL.Cg-Ifngr1^tm1Agt Ifnar1^tm1Agt Fas^lpr

immune system

salivary gland inflammation ( J:92084 )

• mild in some mice

decreased IgG level ( J:92084 )

• modestly compared with Ifngr1^tm1Agt Fas^lpr or Ifnar1^tm1Agt Fas^lpr double homozygotes

increased IgG level ( J:92084 )

• some mice exhibit IgG deposits in the kidney unlike wild-type mice

small lymph nodes ( J:92084 )

• compared with Fas^lpr homozygotes

decreased susceptibility to systemic lupus erythematosus ( J:92084 )

• mice exhibit reduced serum levels of kappa-chain rheumatoid factor and DNA auto-antibodies, lymphadenopathy, glomerulonephritis, renal immunoglobulin deposits, proteinuria, and end organ disease compared with Fas^lpr homozygotes

increased susceptibility to systemic lupus erythematosus ( J:92084 )

• compared with Ifngr1^tm1Agt Fas^lpr double homozygotes

decreased autoantibody level ( J:92084 )

• mice exhibit reduced serum levels of kappa-chain rheumatoid factor and DNA auto-antibodies compared to in Ifngr1^tm1Agt Fas^lpr or Ifnar1^tm1Agt Fas^lpr double homozygotes

decreased anti-nuclear antigen antibody level ( J:92084 )

• compared with Ifngr1^tm1Agt Fas^lpr or Ifnar1^tm1Agt Fas^lpr double homozygotes at 24 weeks

glomerulonephritis ( J:92084 )

• in some mice

renal/urinary system

increased urine protein level ( J:92084 )

• in some mice

glomerulonephritis ( J:92084 )

• in some mice

homeostasis/metabolism

increased urine protein level ( J:92084 )

• in some mice

endocrine/exocrine glands

salivary gland inflammation ( J:92084 )

• mild in some mice

digestive/alimentary system

salivary gland inflammation ( J:92084 )

• mild in some mice

hematopoietic system

decreased IgG level ( J:92084 )

• modestly compared with Ifngr1^tm1Agt Fas^lpr or Ifnar1^tm1Agt Fas^lpr double homozygotes

increased IgG level ( J:92084 )

• some mice exhibit IgG deposits in the kidney unlike wild-type mice