All Phenotypes Ikzf3<tm1Kge> MGI Mouse

Phenotypes associated with this allele

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Ikzf3^tm1Kge/Ikzf3^tm1Kge	involves: 129S4/SvJae	MGI:2653192
hm2	Ikzf3^tm1Kge/Ikzf3^tm1Kge	involves: 129S4/SvJae * C57BL/6	MGI:2653187
cx3	Pou2af1^tm1Pmt/Pou2af1^tm1Pmt Ikzf3^tm1Kge/Ikzf3^tm1Kge	involves: 129P2/OlaHsd * 129S4/SvJae * C57BL/6	MGI:2653193
cx4	Btk^xid/Btk^xid Ikzf3^tm1Kge/Ikzf3^tm1Kge	involves: 129S4/SvJae * CBA/CaHN	MGI:4453853
cx5	Btk^xid/Y Ikzf3^tm1Kge/Ikzf3^tm1Kge	involves: 129S4/SvJae * CBA/CaHN	MGI:4453854

Allelic Composition	Ikzf3^tm1Kge/Ikzf3^tm1Kge
Genetic Background	involves: 129S4/SvJae

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ikzf3^tm1Kge mutation (0 available); any Ikzf3 mutation (30 available)

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

immune system

decreased marginal zone B cell number ( J:69477 )

• numbers of marginal zone B cells and presumed marginal zone B cell precursors are dramatically reduced

increased follicular B cell number ( J:69477 )

• increase in IgD^hi IgM^lo mature follicular B cells

• increase in the ratio of IgD^hi follicular B cells to IgM^hiIgD^loCD21^lo newly formed B cells

hematopoietic system

decreased marginal zone B cell number ( J:69477 )

• numbers of marginal zone B cells and presumed marginal zone B cell precursors are dramatically reduced

increased follicular B cell number ( J:69477 )

• increase in IgD^hi IgM^lo mature follicular B cells

• increase in the ratio of IgD^hi follicular B cells to IgM^hiIgD^loCD21^lo newly formed B cells

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

mortality/aging

premature death ( J:81805 )

• incomplete penetrance, likely due to renal failure from SLE-like disease

neoplasm

increased B cell derived lymphoma incidence ( J:50626 )

• correlated with proliferative phenotype seen in B cells

hematopoietic system

increased B cell proliferation ( J:50626 )

• unimmunized mice at 2 months displayed numerous splenic germinal centers, suggestive of active, proliferative state

• increased B cell proliferation via the immunoglobulin receptor in vitro

increased T cell proliferation ( J:50626 )

• increased T cell receptor mediated proliferation, but T cell development normal

• normal T cell-dependent immune responses, as assessed by antibody titers after immunization with TNP-OVA

enlarged spleen ( J:50626 )

• evident between 3 and 6 months of age

abnormal B cell differentiation ( J:50626 )

decreased B cell number ( J:81805 )

• concomitant 50% decrease in long-lived recirculating bone marrow B cells

increased pre-B cell number ( J:50626 )

• 50% increase in the number of pre-B cells in the bone marrow compared to controls

decreased naive B cell number ( J:81805 )

• small, 17% decrease in mature naive B cells

increased spleen germinal center number ( J:50626 )

• increased numbers of large, well developed germinal centers evident at 6 weeks of age

abnormal spleen marginal zone morphology ( J:50626 )

• ill-defined marginal zone evident at 6 weeks of age

increased IgE level ( J:50626 )

increased IgG1 level ( J:50626 )

increased IgG2a level ( J:50626 )

decreased IgG2b level ( J:50626 )

decreased IgG3 level ( J:50626 )

decreased IgM level ( J:50626 )

homeostasis/metabolism

increased circulating creatinine level ( J:81805 )

• evident in 7 of 24 3 to 5 month old mice

abnormal blood urea nitrogen level ( J:81805 )

• evident in 7 of 24 3 to 5 month old mice

increased urine protein level ( J:81805 )

• incomplete penetrance, evident in several mice, indicative, along with increased creatinine and urea in serum, of renal failure

immune system

increased B cell proliferation ( J:50626 )

• unimmunized mice at 2 months displayed numerous splenic germinal centers, suggestive of active, proliferative state

• increased B cell proliferation via the immunoglobulin receptor in vitro

increased T cell proliferation ( J:50626 )

• increased T cell receptor mediated proliferation, but T cell development normal

• normal T cell-dependent immune responses, as assessed by antibody titers after immunization with TNP-OVA

enlarged spleen ( J:50626 )

• evident between 3 and 6 months of age

abnormal B cell differentiation ( J:50626 )

decreased B cell number ( J:81805 )

• concomitant 50% decrease in long-lived recirculating bone marrow B cells

increased pre-B cell number ( J:50626 )

• 50% increase in the number of pre-B cells in the bone marrow compared to controls

decreased naive B cell number ( J:81805 )

• small, 17% decrease in mature naive B cells

increased spleen germinal center number ( J:50626 )

• increased numbers of large, well developed germinal centers evident at 6 weeks of age

abnormal spleen marginal zone morphology ( J:50626 )

• ill-defined marginal zone evident at 6 weeks of age

increased IgE level ( J:50626 )

increased IgG1 level ( J:50626 )

increased IgG2a level ( J:50626 )

decreased IgG2b level ( J:50626 )

decreased IgG3 level ( J:50626 )

decreased IgM level ( J:50626 )

abnormal type III hypersensitivity reaction ( J:81805 )

• SLE-like phenotype seen

autoimmune response ( J:81805 )

• deposition of IgG, IgM, and C3 in glomeruli

increased autoantibody level ( J:50626 , J:81805 )

• autoantibodies reactive with kidney sections of wild-type mice were detected in 5 or 6 week old mutant mice, but not in controls (J:50626)

• increased autoantibody (anti-ANA, anti-ssDNA, anti-dsDNA, anti-histone) concentrations in serum, especially in female mice (J:81805)

increased anti-nuclear antigen antibody level ( J:81805 )

increased anti-double stranded DNA antibody level ( J:81805 )

increased anti-histone antibody level ( J:81805 )

increased anti-single stranded DNA antibody level ( J:81805 )

glomerulonephritis ( J:81805 )

• hypercellularity, lobularity, segmental sclerosis, and enlarged glomeruli, caused by immune complex deposits

renal/urinary system

increased urine protein level ( J:81805 )

• incomplete penetrance, evident in several mice, indicative, along with increased creatinine and urea in serum, of renal failure

glomerulonephritis ( J:81805 )

• hypercellularity, lobularity, segmental sclerosis, and enlarged glomeruli, caused by immune complex deposits

renal glomerular immunoglobulin deposits ( J:81805 )

cellular

increased B cell proliferation ( J:50626 )

• unimmunized mice at 2 months displayed numerous splenic germinal centers, suggestive of active, proliferative state

• increased B cell proliferation via the immunoglobulin receptor in vitro

increased T cell proliferation ( J:50626 )

• increased T cell receptor mediated proliferation, but T cell development normal

• normal T cell-dependent immune responses, as assessed by antibody titers after immunization with TNP-OVA

growth/size/body

enlarged spleen ( J:50626 )

• evident between 3 and 6 months of age

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
systemic lupus erythematosus		DOID:9074	OMIM:152700 OMIM:300809 OMIM:605480 OMIM:608437 OMIM:609903 OMIM:609939 OMIM:610065 OMIM:610066 OMIM:612254 OMIM:612378 OMIM:613145 OMIM:614420	J:50626 , J:81805

Allelic Composition	Pou2af1^tm1Pmt/Pou2af1^tm1Pmt Ikzf3^tm1Kge/Ikzf3^tm1Kge
Genetic Background	involves: 129P2/OlaHsd * 129S4/SvJae * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ikzf3^tm1Kge mutation (0 available); any Ikzf3 mutation (30 available)
Pou2af1^tm1Pmt mutation (0 available); any Pou2af1 mutation (15 available)

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

hematopoietic system

arrested B cell differentiation ( J:81805 )

• blocked transition from pre-B to immature B cells

absent spleen germinal center ( J:81805 )

• failed to form germinal centers

decreased immunoglobulin level ( J:81805 )

• of all types, relative to wild-type controls

• no development of autoantibodies as seen in Zfpn1a3^tm1Kge mutant mice

decreased IgA level ( J:81805 )

decreased IgD level ( J:81805 )

decreased IgE level ( J:81805 )

decreased IgG level ( J:81805 )

decreased IgM level ( J:81805 )

immune system

arrested B cell differentiation ( J:81805 )

• blocked transition from pre-B to immature B cells

absent spleen germinal center ( J:81805 )

• failed to form germinal centers

decreased immunoglobulin level ( J:81805 )

• of all types, relative to wild-type controls

• no development of autoantibodies as seen in Zfpn1a3^tm1Kge mutant mice

decreased IgA level ( J:81805 )

decreased IgD level ( J:81805 )

decreased IgE level ( J:81805 )

decreased IgG level ( J:81805 )

decreased IgM level ( J:81805 )

renal/urinary system

renal/urinary system phenotype ( J:81805 )

N	• histological examination of kidneys showed no inflammation, no glomerular immune complex deposition

Allelic Composition	Btk^xid/Btk^xid Ikzf3^tm1Kge/Ikzf3^tm1Kge
Genetic Background	involves: 129S4/SvJae * CBA/CaHN

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Btk^xid mutation (3 available); any Btk mutation (21 available)
Ikzf3^tm1Kge mutation (0 available); any Ikzf3 mutation (30 available)

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

hematopoietic system

decreased follicular B cell number ( J:69477 )

• decrease in IgD^hi IgM^lo mature follicular B cells, similar to that seen in single homozygous Btk mutant mice

• however, normal numbers of marginal zone B cells are observed

immune system

decreased follicular B cell number ( J:69477 )

• decrease in IgD^hi IgM^lo mature follicular B cells, similar to that seen in single homozygous Btk mutant mice

• however, normal numbers of marginal zone B cells are observed

Allelic Composition	Btk^xid/Y Ikzf3^tm1Kge/Ikzf3^tm1Kge
Genetic Background	involves: 129S4/SvJae * CBA/CaHN

Find Mice

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

hematopoietic system

decreased follicular B cell number ( J:69477 )

• decrease in IgD^hi IgM^lo mature follicular B cells, similar to that seen in single homozygous Btk mutant mice

• however, normal numbers of marginal zone B cells are observed

immune system

decreased follicular B cell number ( J:69477 )

• decrease in IgD^hi IgM^lo mature follicular B cells, similar to that seen in single homozygous Btk mutant mice

• however, normal numbers of marginal zone B cells are observed

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