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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tnfrsf9tm1Byk
targeted mutation 1, Byoung Kwon
MGI:2664914
Summary 4 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
hm1
Tnfrsf9tm1Byk/Tnfrsf9tm1Byk B6.Cg-Tnfrsf9tm1Byk MGI:2664915
hm2
Tnfrsf9tm1Byk/Tnfrsf9tm1Byk C.Cg-Tnfrsf9tm1Byk MGI:4441173
cx3
Cd28tm1Pjl/Cd28tm1Pjl
Tnfrsf9tm1Byk/Tnfrsf9tm1Byk
either: B6.Cg-Cd28tm1Pjl Tnfrsf9tm1Byk or C.Cg-Cd28tm1Pjl Tnfrsf9tm1Byk MGI:2664937
cx4
Faslpr/Faslpr
Tnfrsf9tm1Byk/Tnfrsf9tm1Byk
MRL.Cg-Tnfrsf9tm1Byk Faslpr MGI:3800222


Genotype
MGI:2664915
hm1
Allelic
Composition
Tnfrsf9tm1Byk/Tnfrsf9tm1Byk
Genetic
Background
B6.Cg-Tnfrsf9tm1Byk
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tnfrsf9tm1Byk mutation (2 available); any Tnfrsf9 mutation (16 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
hematopoietic system
• absolute numbers of granulocyte-macrophage, erythroid, and multipotential progenitor cells in the bone marrow, blood, and spleen are higher in mutants than wild-type mice
• 2-3-fold decrease in IgG2a in response to keyhole limpet hemocyanin (KLH) immunization
• 2-3-fold decrease in IgG3 in response to KLH immunization
• mutants show significantly decreased cytotoxic T lymphocyte activity to vesicular stomatitis virus
• proliferation of splenocytes/T-cells is increased compared to wild-type mice when stimulated with anti-CD3 mAb and ConA

immune system
• 2-3-fold decrease in IgG2a in response to keyhole limpet hemocyanin (KLH) immunization
• 2-3-fold decrease in IgG3 in response to KLH immunization
• mutants show significantly decreased cytotoxic T lymphocyte activity to vesicular stomatitis virus
• proliferation of splenocytes/T-cells is increased compared to wild-type mice when stimulated with anti-CD3 mAb and ConA
• decrease in IFN-gamma production in response to KLH immunization
• moderate decrease in IFN-gamma secretion from T cells
• moderate decrease in IL-2 secretion from T cells
• significant decrease in IL-4 secretion from T cells




Genotype
MGI:4441173
hm2
Allelic
Composition
Tnfrsf9tm1Byk/Tnfrsf9tm1Byk
Genetic
Background
C.Cg-Tnfrsf9tm1Byk
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tnfrsf9tm1Byk mutation (2 available); any Tnfrsf9 mutation (16 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
hematopoietic system
• splenocytes show increased cellular proliferation compared to wild-type mice when stimulated with anti-CD3 or ConA
• absolute numbers of granulocyte-macrophage, erythroid, and multipotential progenitor cells in the bone marrow, blood, and spleen are higher in mutants than wild-type mice
• in naive mice
• 2-3-fold decrease in IgG2a in response to keyhole limpet hemocyanin (KLH) immunization
• in naive mice
• in naive mice
• 2-3-fold decrease in IgG3 in response to KLH immunization
• Vesicular stomatitis virus-specific cytotoxic T lymphocyte responses and plaque reduction neutralizing ability is reduced
• proliferation of splenocytes/T-cells is increased compared to wild-type mice when stimulated with anti-CD3 mAb and ConA

immune system
• splenocytes show increased cellular proliferation compared to wild-type mice when stimulated with anti-CD3 or ConA
• in naive mice
• 2-3-fold decrease in IgG2a in response to keyhole limpet hemocyanin (KLH) immunization
• in naive mice
• in naive mice
• 2-3-fold decrease in IgG3 in response to KLH immunization
• Vesicular stomatitis virus-specific cytotoxic T lymphocyte responses and plaque reduction neutralizing ability is reduced
• proliferation of splenocytes/T-cells is increased compared to wild-type mice when stimulated with anti-CD3 mAb and ConA
• decrease in IFN-gamma production in response to KLH immunization
• moderate decrease in IFN-gamma secretion from T cells
• following ConA stimulation
• moderate decrease in IL-2 secretion from T cells
• significant decrease in IL-4 secretion from T cells

cellular
• splenocytes show increased cellular proliferation compared to wild-type mice when stimulated with anti-CD3 or ConA




Genotype
MGI:2664937
cx3
Allelic
Composition
Cd28tm1Pjl/Cd28tm1Pjl
Tnfrsf9tm1Byk/Tnfrsf9tm1Byk
Genetic
Background
either: B6.Cg-Cd28tm1Pjl Tnfrsf9tm1Byk or C.Cg-Cd28tm1Pjl Tnfrsf9tm1Byk
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cd28tm1Pjl mutation (0 available); any Cd28 mutation (19 available)
Tnfrsf9tm1Byk mutation (2 available); any Tnfrsf9 mutation (16 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
immune system
• splenocytes show decreased cellular proliferation compared to wild-type mice when stimulated with anti-CD3 or ConA
• mutants are incapable of undergoing Ig class switch and fail to mount anti-DNP responses of IgG1, IgG2a and IgM
• naive mutants exhibit decreased IgG1 levels and fail to mount anti-DNP responses of IgG1
• naive mutants exhibit decreased IgG2a levels and fail to mount anti-DNP responses of IgG2a
• mutants fail to mount anti-DNP responses of IgM and secrete reduced levels of IgM in response to a TD-antigen
• Vesicular stomatitis virus-specific cytotoxic T lymphocyte responses and plaque reduction neutralizing ability is reduced
• mutants exhibit significantly suppressed contact hypersensitivity to FITC and DNFB
• following ConA stimulation

hematopoietic system
• splenocytes show decreased cellular proliferation compared to wild-type mice when stimulated with anti-CD3 or ConA
• mutants are incapable of undergoing Ig class switch and fail to mount anti-DNP responses of IgG1, IgG2a and IgM
• naive mutants exhibit decreased IgG1 levels and fail to mount anti-DNP responses of IgG1
• naive mutants exhibit decreased IgG2a levels and fail to mount anti-DNP responses of IgG2a
• mutants fail to mount anti-DNP responses of IgM and secrete reduced levels of IgM in response to a TD-antigen
• Vesicular stomatitis virus-specific cytotoxic T lymphocyte responses and plaque reduction neutralizing ability is reduced

cellular
• splenocytes show decreased cellular proliferation compared to wild-type mice when stimulated with anti-CD3 or ConA




Genotype
MGI:3800222
cx4
Allelic
Composition
Faslpr/Faslpr
Tnfrsf9tm1Byk/Tnfrsf9tm1Byk
Genetic
Background
MRL.Cg-Tnfrsf9tm1Byk Faslpr
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Faslpr mutation (24 available); any Fas mutation (54 available)
Tnfrsf9tm1Byk mutation (2 available); any Tnfrsf9 mutation (16 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cellular
• more apoptotic cells are detected relative to wild-type Tnfrsf9, Fas lpr controls

mortality/aging
• by 5 months, 80% mortality is observed compared to 40% in Tnfrsf9-sufficient, Fas-null mice; by 4 months, mice become increasingly moribund and display reduced activity

hematopoietic system
N
• CD8beta+ T cells are not altered in number at any age
• in lacrimal glands, DN T cell percentages are about 2-fold greater than in wild-type Tnfrsf9, Fas lpr controls; similar increases are seen in spleen and salivary glands (J:126929)
• increased proportion seen in spleen compared to Tnfrsf9-sufficient, Fas-null mice (J:127197)
• percentages of B-1 (B220+ CD5+) cells are increased significantly in peritoneums and slightly higher in spleens
• percentages of B-2( B220+ CD5-) cells are increased significantly in peritoneums and slightly higher in spleens
• 3- and 5-month old mice show elevated CD4+ T cell numbers in lacrimal glands (J:126929)
• mice display early increases in CD4+ T cells in spleen, and numbers keep rising (J:127197)
• significant enlargement of axillary lymph nodes is seen at 5 months compared to Tnfrsf9-wt, Fas-null mice
• greatly increased germinal center formation is observed compared to Tnfrsf9-sufficient, Fas-null mice
• circulating titres are elevated compared with compared to Tnfrsf9-sufficient, Fas-null mice

immune system
• extensive and early inflammation is observed compared with control wild-type Tnfrsf9, Fas lpr homozygotes
• by 3 months, extensive mononuclear cell infiltration is observed, becoming intense by 5 months while controls show no infiltrate at 1 month, mimimal inflammatory lesions at 3 months and extensive, although less severe than double mutants, infiltrates by 5 months of age
• in lacrimal glands, DN T cell percentages are about 2-fold greater than in wild-type Tnfrsf9, Fas lpr controls; similar increases are seen in spleen and salivary glands (J:126929)
• increased proportion seen in spleen compared to Tnfrsf9-sufficient, Fas-null mice (J:127197)
• percentages of B-1 (B220+ CD5+) cells are increased significantly in peritoneums and slightly higher in spleens
• percentages of B-2( B220+ CD5-) cells are increased significantly in peritoneums and slightly higher in spleens
• 3- and 5-month old mice show elevated CD4+ T cell numbers in lacrimal glands (J:126929)
• mice display early increases in CD4+ T cells in spleen, and numbers keep rising (J:127197)
• significant enlargement of axillary lymph nodes is seen at 5 months compared to Tnfrsf9-wt, Fas-null mice
• greatly increased germinal center formation is observed compared to Tnfrsf9-sufficient, Fas-null mice
• circulating titres are elevated compared with compared to Tnfrsf9-sufficient, Fas-null mice
• significant enlargement at 5 months compared to Tnfrsf9-wt, Fas-null mice
• in lacrimal glands, increased levels of Il-4 are detected compared to controls
• phenotypic manifestations are increased relative to Faslpr homozygotes
• serum titres of anti-nuclear IgG1/2a are increased at 3 and 5 months
• anti-DNA antibody production is increased compared to Tnfrsf9-sufficient, Fas-null mice

renal/urinary system
• increased immunoglobulin deposits are detected in kidneys compared to controls
• mice show exacerbated renal injury, with increased glomerular infiltrates
• IgG and C3 depositions in kidneys are increased compared with Tnfrsf9-sufficient, Fas-null mice

craniofacial
• from 4 months of age, >60% of mice display progressive erosion of pinnae

hearing/vestibular/ear
• from 4 months of age, >60% of mice display progressive erosion of pinnae

endocrine/exocrine glands
• more apoptotic cells are detected relative to wild-type Tnfrsf9, Fas lpr controls
• increased deposits of IgG1, but not IgG2a are detected in lacrimal glands at 3 and 5 months
• extensive and early inflammation is observed compared with control wild-type Tnfrsf9, Fas lpr homozygotes
• by 3 months, extensive mononuclear cell infiltration is observed, becoming intense by 5 months while controls show no infiltrate at 1 month, mimimal inflammatory lesions at 3 months and extensive, although less severe than double mutants, infiltrates by 5 months of age

vision/eye
• more apoptotic cells are detected relative to wild-type Tnfrsf9, Fas lpr controls
• increased deposits of IgG1, but not IgG2a are detected in lacrimal glands at 3 and 5 months
• extensive and early inflammation is observed compared with control wild-type Tnfrsf9, Fas lpr homozygotes
• by 3 months, extensive mononuclear cell infiltration is observed, becoming intense by 5 months while controls show no infiltrate at 1 month, mimimal inflammatory lesions at 3 months and extensive, although less severe than double mutants, infiltrates by 5 months of age

integument
• by 5 months, skin lesions around tip of nose and around ears are more pronounced than in Tnfrsf9-wt, Fas-null mice

growth/size/body
• from 4 months of age, >60% of mice display progressive erosion of pinnae

Mouse Models of Human Disease
OMIM ID Ref(s)
Sjogren Syndrome 270150 J:120559
Systemic Lupus Erythematosus; SLE 152700 J:120559





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last database update
11/29/2016
MGI 6.06
The Jackson Laboratory