Phenotypes associated with this allele

• Allele Symbol: Ikzf1^tm1Kge
• Allele Name: targeted mutation 1, Katia Georgopoulos
• Allele ID: MGI:2158738
• Summary: 4 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Ikzf1^tm1Kge/Ikzf1^tm1Kge	involves: 129S4/SvJae * BALB/c	MGI:4360637
hm2	Ikzf1^tm1Kge/Ikzf1^tm1Kge	involves: 129S4/SvJae * C57BL/6	MGI:4360636
ht3	Ikzf1^tm1Kge/Ikzf1^+	involves: 129S4/SvJae * BALB/c	MGI:4360784
ht4	Ikzf1^tm1Kge/Ikzf1^+	involves: 129S4/SvJae * C57BL/6	MGI:4360783

Genotype
MGI:4360637

hm1

• Allelic Composition: Ikzf1^tm1Kge/Ikzf1^tm1Kge
• Genetic Background: involves: 129S4/SvJae * BALB/c

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

mortality/aging

postnatal lethality, incomplete penetrance ( J:20671 )

• 95% of mice die by 4 weeks of age due to bacterial infections when housed under standard conditions

growth/size/body

decreased body size ( J:20671 )

• mice are one-third to two-thirds smaller than controls at one week of age

enlarged spleen ( J:20671 )

• spleens of mice are 1.5 to 3 times larger than controls at 2 weeks of age

hematopoietic system

abnormal thymus morphology ( J:20671 )

abnormal thymus cortex morphology ( J:20671 )

• only a rudimentary thymus with few lymphocytes found in the cortex

thymus hypoplasia ( J:20671 )

• thymus cellularity in most mice is about 1000-fold less than controls

• in 5% of mice, a larger thymus is present

abnormal definitive hematopoiesis ( J:20671 )

abnormal common myeloid progenitor cell morphology ( J:20671 )

• 9% of splenocytes in young mice and 60% of splenocytes in older mice are myeloid precursors while control spleens only have about 5% myeloid precursors

• cell differentiation assays confirm 10-fold more macrophage and granulocytes can be derived from mutant spleen than control spleen

arrested T cell differentiation ( J:20671 )

• in 95% of mice, the few thymocytes present appear to be arrested in the double-positive stage

extramedullary hematopoiesis ( J:20671 )

• erythroid precursors make up 70% of splenocytes at two weeks of age and over 25% of splenocytes in older mice

decreased bone marrow cell number ( J:20671 )

• bone marrow cellularity is reduced 10-fold compared to controls

abnormal erythroid progenitor cell morphology ( J:20671 )

• ratio of erythroid precursors to myeloid precursors is twice that of controls in two week old bone marrow

• the ratio of erythroid precursors to myeloid precursors drops from 2:1 to 1:3 with age while in controls this ratio goes from 1:1 to 1:2

increased basophil cell number ( J:20671 )

• increased numbers of basophilic cells are observed in the blood

abnormal eosinophil morphology ( J:20671 )

• a large number and variety of polymorphonuclear cells are found in the blood

increased nucleated erythrocyte cell number ( J:20671 )

• nucleated erythrocytes are found in the blood of both young and old mice

absent B cells ( J:20671 )

• B cells are absent in these mice including pre-B cells in the bone marrow

absent pre-B cells ( J:20671 )

• are absent in the bone marrow

decreased gamma-delta intraepithelial T cell number ( J:20671 )

• dendritic epithelial gamma-delta T cells are absent from the skin

abnormal NK cell morphology ( J:20671 )

• NK cells are absent in the spleen

abnormal spleen morphology ( J:20671 )

enlarged spleen ( J:20671 )

• spleens of mice are 1.5 to 3 times larger than controls at 2 weeks of age

intermingled spleen red and white pulp ( J:20671 )

• the well structured red and white pulp architecture of the spleen is disrupted in these mice

• spleen consists of myeloid and erythrocyte precursors instead of lymphocytes and mature erythrocytes

immune system

arrested T cell differentiation ( J:20671 )

• in 95% of mice, the few thymocytes present appear to be arrested in the double-positive stage

increased basophil cell number ( J:20671 )

• increased numbers of basophilic cells are observed in the blood

abnormal eosinophil morphology ( J:20671 )

• a large number and variety of polymorphonuclear cells are found in the blood

absent B cells ( J:20671 )

• B cells are absent in these mice including pre-B cells in the bone marrow

absent pre-B cells ( J:20671 )

• are absent in the bone marrow

decreased gamma-delta intraepithelial T cell number ( J:20671 )

• dendritic epithelial gamma-delta T cells are absent from the skin

abnormal NK cell morphology ( J:20671 )

• NK cells are absent in the spleen

abnormal immune system organ morphology ( J:20671 )

abnormal thymus morphology ( J:20671 )

abnormal thymus cortex morphology ( J:20671 )

• only a rudimentary thymus with few lymphocytes found in the cortex

thymus hypoplasia ( J:20671 )

• thymus cellularity in most mice is about 1000-fold less than controls

• in 5% of mice, a larger thymus is present

abnormal spleen morphology ( J:20671 )

enlarged spleen ( J:20671 )

• spleens of mice are 1.5 to 3 times larger than controls at 2 weeks of age

intermingled spleen red and white pulp ( J:20671 )

• the well structured red and white pulp architecture of the spleen is disrupted in these mice

• spleen consists of myeloid and erythrocyte precursors instead of lymphocytes and mature erythrocytes

absent Peyer's patches ( J:20671 )

• Peyer's patches are absent in these mice

absent mesenteric lymph nodes ( J:20671 )

absent axillary lymph nodes ( J:20671 )

absent cervical lymph nodes ( J:20671 )

absent inguinal lymph nodes ( J:20671 )

lung inflammation ( J:20671 )

• pneumonia results from bacterial infections that occur under standard housing conditions

• pneumonia is associated with abscesses, large number of bacteria, and infiltration of myeloid cells

increased susceptibility to bacterial infection ( J:20671 )

• mouse housed under normal conditions die of bacterial infections

• Enterobacteria species are found in focal lesions of the liver, in abcesses in the lung, and systemically in the blood

liver/biliary system

multifocal hepatic necrosis ( J:20671 )

• focal infarcts result from bacterial infections that occur under standard housing conditions

• infarcts appear as white nodules

• large number of macrophages and eosinophils are evident in areas of infection

• sometimes necrosis takes up half the liver

respiratory system

lung inflammation ( J:20671 )

• pneumonia results from bacterial infections that occur under standard housing conditions

• pneumonia is associated with abscesses, large number of bacteria, and infiltration of myeloid cells

digestive/alimentary system

abnormal gut flora balance ( J:20671 )

• the intestinal flora contains more numbers and more diverse groups of bacteria than in controls

endocrine/exocrine glands

abnormal thymus morphology ( J:20671 )

abnormal thymus cortex morphology ( J:20671 )

• only a rudimentary thymus with few lymphocytes found in the cortex

thymus hypoplasia ( J:20671 )

• thymus cellularity in most mice is about 1000-fold less than controls

• in 5% of mice, a larger thymus is present

Genotype
MGI:4360636

hm2

• Allelic Composition: Ikzf1^tm1Kge/Ikzf1^tm1Kge
• Genetic Background: involves: 129S4/SvJae * C57BL/6

mortality/aging

postnatal lethality, incomplete penetrance ( J:20671 )

• 95% of mice die by 4 weeks of age due to bacterial infections when housed under standard conditions

growth/size/body

decreased body size ( J:20671 )

• mice are one-third to two-thirds smaller than controls at one week of age

enlarged spleen ( J:20671 )

• spleens of mice are 1.5 to 3 times larger than controls at 2 weeks of age

hematopoietic system

abnormal thymus morphology ( J:20671 )

abnormal thymus cortex morphology ( J:20671 )

• only a rudimentary thymus with few lymphocytes found in the cortex

thymus hypoplasia ( J:20671 )

• thymus cellularity in most mice is about 1000-fold less than controls

• in 5% of mice, a larger thymus is present

abnormal definitive hematopoiesis ( J:20671 )

abnormal common myeloid progenitor cell morphology ( J:20671 )

• 9% of splenocytes in young mice and 60% of splenocytes in older mice are myeloid precursors while control spleens only have about 5% myeloid precursors

• cell differentiation assays confirm 10-fold more macrophage and granulocytes can be derived from mutant spleen than control spleen

arrested T cell differentiation ( J:20671 )

• in 95% of mice, the few thymocytes present appear to be arrested in the double-positive stage

extramedullary hematopoiesis ( J:20671 )

• erythroid precursors make up 70% of splenocytes at two weeks of age and over 25% of splenocytes in older mice

decreased bone marrow cell number ( J:20671 )

• bone marrow cellularity is reduced 10-fold compared to controls

abnormal erythroid progenitor cell morphology ( J:20671 )

• ratio of erythroid precursors to myeloid precursors is twice that of controls in two week old bone marrow

• the ratio of erythroid precursors to myeloid precursors drops from 2:1 to 1:3 with age while in controls this ratio goes from 1:1 to 1:2

increased basophil cell number ( J:20671 )

• increased numbers of basophilic cells are observed in the blood

abnormal eosinophil morphology ( J:20671 )

• a large number and variety of polymorphonuclear cells are found in the blood

increased nucleated erythrocyte cell number ( J:20671 )

• nucleated erythrocytes are found in the blood of both young and old mice

absent B cells ( J:20671 )

• B cells are absent in these mice including pre-B cells in the bone marrow

absent pre-B cells ( J:20671 )

• are absent in the bone marrow

decreased gamma-delta intraepithelial T cell number ( J:20671 )

• dendritic epithelial gamma-delta T cells are absent from the skin

abnormal NK cell morphology ( J:20671 )

• NK cells are absent in the spleen

abnormal spleen morphology ( J:20671 )

enlarged spleen ( J:20671 )

• spleens of mice are 1.5 to 3 times larger than controls at 2 weeks of age

intermingled spleen red and white pulp ( J:20671 )

• the well structured red and white pulp architecture of the spleen is disrupted in these mice

• spleen consists of myeloid and erythrocyte precursors instead of lymphocytes and mature erythrocytes

immune system

arrested T cell differentiation ( J:20671 )

• in 95% of mice, the few thymocytes present appear to be arrested in the double-positive stage

increased basophil cell number ( J:20671 )

• increased numbers of basophilic cells are observed in the blood

abnormal eosinophil morphology ( J:20671 )

• a large number and variety of polymorphonuclear cells are found in the blood

absent B cells ( J:20671 )

• B cells are absent in these mice including pre-B cells in the bone marrow

absent pre-B cells ( J:20671 )

• are absent in the bone marrow

decreased gamma-delta intraepithelial T cell number ( J:20671 )

• dendritic epithelial gamma-delta T cells are absent from the skin

abnormal NK cell morphology ( J:20671 )

• NK cells are absent in the spleen

abnormal immune system organ morphology ( J:20671 )

abnormal thymus morphology ( J:20671 )

abnormal thymus cortex morphology ( J:20671 )

• only a rudimentary thymus with few lymphocytes found in the cortex

thymus hypoplasia ( J:20671 )

• thymus cellularity in most mice is about 1000-fold less than controls

• in 5% of mice, a larger thymus is present

abnormal spleen morphology ( J:20671 )

enlarged spleen ( J:20671 )

• spleens of mice are 1.5 to 3 times larger than controls at 2 weeks of age

intermingled spleen red and white pulp ( J:20671 )

• the well structured red and white pulp architecture of the spleen is disrupted in these mice

• spleen consists of myeloid and erythrocyte precursors instead of lymphocytes and mature erythrocytes

absent Peyer's patches ( J:20671 )

• Peyer's patches are absent in these mice

absent mesenteric lymph nodes ( J:20671 )

absent axillary lymph nodes ( J:20671 )

absent cervical lymph nodes ( J:20671 )

absent inguinal lymph nodes ( J:20671 )

lung inflammation ( J:20671 )

• pneumonia results from bacterial infections that occur under standard housing conditions

• pneumonia is associated with abscesses, large number of bacteria, and infiltration of myeloid cells

increased susceptibility to bacterial infection ( J:20671 )

• mouse housed under normal conditions die of bacterial infections

• Enterobacteria species are found in focal lesions of the liver, in abcesses in the lung, and systemically in the blood

liver/biliary system

multifocal hepatic necrosis ( J:20671 )

• focal infarcts result from bacterial infections that occur under standard housing conditions

• infarcts appear as white nodules

• large number of macrophages and eosinophils are evident in areas of infection

• sometimes necrosis takes up half the liver

respiratory system

lung inflammation ( J:20671 )

• pneumonia results from bacterial infections that occur under standard housing conditions

• pneumonia is associated with abscesses, large number of bacteria, and infiltration of myeloid cells

digestive/alimentary system

abnormal gut flora balance ( J:20671 )

• the intestinal flora contains more numbers and more diverse groups of bacteria than in controls

endocrine/exocrine glands

abnormal thymus morphology ( J:20671 )

abnormal thymus cortex morphology ( J:20671 )

• only a rudimentary thymus with few lymphocytes found in the cortex

thymus hypoplasia ( J:20671 )

• thymus cellularity in most mice is about 1000-fold less than controls

• in 5% of mice, a larger thymus is present

Genotype
MGI:4360784

ht3

• Allelic Composition: Ikzf1^tm1Kge/Ikzf1⁺
• Genetic Background: involves: 129S4/SvJae * BALB/c

mortality/aging

premature death ( J:29355 )

• mice develop a fatal leukemia between 3 and 6 months of age

neoplasm

increased leukemia incidence ( J:29355 )

• leukemia derived from alpha-beta T cells occurs in all mice

• the cells are large, can be either CD4⁺ or CD8⁺, and are monoclonal in their use of TCR beta chains

• these lymphoblastic cells accumulate in all immune organs and in the liver, kidney, and lung

• leukemia cells disrupt the architecture of numerous organs as the mice age

• studies with 2-month old mice suggest the malignant cells arise in the thymus and not in the spleen

immune system

increased T cell proliferation ( J:29355 )

• basal proliferation of T cells is 2.6 fold higher than controls

abnormal thymus lobule morphology ( J:29355 )

• leukemia leads to the loss of the bi-lobed structure of the thymus in mice over 3 months of age

thymus cortex hypoplasia ( J:29355 )

• leukemia in older mice leads to complete effacement of cortical and medullary structures in the thymus

abnormal thymus medulla morphology ( J:29355 )

• leukemia in older mice leads to complete effacement of cortical and medullary structures in the thymus

enlarged thymus ( J:29355 )

• leukemia leads to a swelling of the thymus in mice over 3 months of age

abnormal thymocyte activation ( J:29355 )

• thymocytes from 1 month old mice have a 200-fold increase in their proliferation rate in response to TCR stimulus compared to controls

• a less dramatic increase in proliferation rate is observed with thymocytes from E18.5 fetuses

spleen hyperplasia ( J:29355 )

• splenocyte numbers are 2- to 5- fold greater than controls by 3 months of age

• leukemia later in life leads to spleen cellularity that is 10-fold greater than normal

abnormal T cell differentiation ( J:29355 )

• starting at ages 2-3 months, intermediate single positive thymoyctes start accumulating

decreased spleen red pulp amount ( J:29355 )

• leukemia leads to an decrease in the red pulp area of the spleen

increased spleen white pulp amount ( J:29355 )

• leukemia leads to an increase in the white pulp area of the spleen

enlarged mesenteric lymph nodes ( J:29355 )

• leukemia results in enlarged lymph nodes for all mice over 3 months of age

• lymph nodes enlarge 20- to 50- fold greater than normal

abnormal lymph node cortex morphology ( J:29355 )

• leukemia leads to the loss of lymph node structure

abnormal lymph node medulla morphology ( J:29355 )

• leukemia leads to the loss of lymph node structure

lymph node hyperplasia ( J:29355 )

• leukemia results in enlarged lymph nodes for all mice over 3 months of age

• lymph nodes enlarge 20- to 50- fold greater than normal

enlarged cervical lymph nodes ( J:29355 )

• leukemia results in enlarged lymph nodes for all mice over 3 months of age

• lymph nodes enlarge 20- to 50- fold greater than normal

hematopoietic system

increased T cell proliferation ( J:29355 )

• basal proliferation of T cells is 2.6 fold higher than controls

abnormal thymus lobule morphology ( J:29355 )

• leukemia leads to the loss of the bi-lobed structure of the thymus in mice over 3 months of age

thymus cortex hypoplasia ( J:29355 )

• leukemia in older mice leads to complete effacement of cortical and medullary structures in the thymus

abnormal thymus medulla morphology ( J:29355 )

• leukemia in older mice leads to complete effacement of cortical and medullary structures in the thymus

enlarged thymus ( J:29355 )

• leukemia leads to a swelling of the thymus in mice over 3 months of age

abnormal thymocyte activation ( J:29355 )

• thymocytes from 1 month old mice have a 200-fold increase in their proliferation rate in response to TCR stimulus compared to controls

• a less dramatic increase in proliferation rate is observed with thymocytes from E18.5 fetuses

spleen hyperplasia ( J:29355 )

• splenocyte numbers are 2- to 5- fold greater than controls by 3 months of age

• leukemia later in life leads to spleen cellularity that is 10-fold greater than normal

abnormal T cell differentiation ( J:29355 )

• starting at ages 2-3 months, intermediate single positive thymoyctes start accumulating

decreased spleen red pulp amount ( J:29355 )

• leukemia leads to an decrease in the red pulp area of the spleen

increased spleen white pulp amount ( J:29355 )

• leukemia leads to an increase in the white pulp area of the spleen

endocrine/exocrine glands

abnormal thymus lobule morphology ( J:29355 )

• leukemia leads to the loss of the bi-lobed structure of the thymus in mice over 3 months of age

thymus cortex hypoplasia ( J:29355 )

• leukemia in older mice leads to complete effacement of cortical and medullary structures in the thymus

abnormal thymus medulla morphology ( J:29355 )

• leukemia in older mice leads to complete effacement of cortical and medullary structures in the thymus

enlarged thymus ( J:29355 )

• leukemia leads to a swelling of the thymus in mice over 3 months of age

abnormal thymocyte activation ( J:29355 )

• thymocytes from 1 month old mice have a 200-fold increase in their proliferation rate in response to TCR stimulus compared to controls

• a less dramatic increase in proliferation rate is observed with thymocytes from E18.5 fetuses

cellular

increased T cell proliferation ( J:29355 )

• basal proliferation of T cells is 2.6 fold higher than controls

growth/size/body

spleen hyperplasia ( J:29355 )

• splenocyte numbers are 2- to 5- fold greater than controls by 3 months of age

• leukemia later in life leads to spleen cellularity that is 10-fold greater than normal

Genotype
MGI:4360783

ht4

• Allelic Composition: Ikzf1^tm1Kge/Ikzf1⁺
• Genetic Background: involves: 129S4/SvJae * C57BL/6

mortality/aging

premature death ( J:29355 )

• mice develop a fatal leukemia between 3 and 6 months of age

neoplasm

increased leukemia incidence ( J:29355 )

• leukemia derived from alpha-beta T cells occurs in all mice

• the cells are large, can be either CD4⁺ or CD8⁺, and are monoclonal in their use of TCR beta chains

• these lymphoblastic cells accumulate in all immune organs and in the liver, kidney, and lung

• leukemia cells disrupt the architecture of numerous organs as the mice age

• studies with 2-month old mice suggest the malignant cells arise in the thymus and not in the spleen

immune system

increased T cell proliferation ( J:29355 )

• basal proliferation of T cells is 2.6 fold higher than controls

abnormal thymus lobule morphology ( J:29355 )

• leukemia leads to the loss of the bi-lobed structure of the thymus in mice over 3 months of age

thymus cortex hypoplasia ( J:29355 )

• leukemia in older mice leads to complete effacement of cortical and medullary structures in the thymus

abnormal thymus medulla morphology ( J:29355 )

• leukemia in older mice leads to complete effacement of cortical and medullary structures in the thymus

enlarged thymus ( J:29355 )

• leukemia leads to a swelling of the thymus in mice over 3 months of age

abnormal thymocyte activation ( J:29355 )

• thymocytes from 1 month old mice have a 200-fold increase in their proliferation rate in response to TCR stimulus compared to controls

• a less dramatic increase in proliferation rate is observed with thymocytes from E18.5 fetuses

spleen hyperplasia ( J:29355 )

• splenocyte numbers are 2- to 5- fold greater than controls by 3 months of age

• leukemia later in life leads to spleen cellularity that is 10-fold greater than normal

abnormal T cell differentiation ( J:29355 )

• starting at ages 2-3 months, intermediate single positive thymoyctes start accumulating

decreased spleen red pulp amount ( J:29355 )

• leukemia leads to an decrease in the red pulp area of the spleen

increased spleen white pulp amount ( J:29355 )

• leukemia leads to an increase in the white pulp area of the spleen

enlarged mesenteric lymph nodes ( J:29355 )

• leukemia results in enlarged lymph nodes for all mice over 3 months of age

• lymph nodes enlarge 20- to 50- fold greater than normal

abnormal lymph node cortex morphology ( J:29355 )

• leukemia leads to the loss of lymph node structure

abnormal lymph node medulla morphology ( J:29355 )

• leukemia leads to the loss of lymph node structure

lymph node hyperplasia ( J:29355 )

• leukemia results in enlarged lymph nodes for all mice over 3 months of age

• lymph nodes enlarge 20- to 50- fold greater than normal

enlarged cervical lymph nodes ( J:29355 )

• leukemia results in enlarged lymph nodes for all mice over 3 months of age

• lymph nodes enlarge 20- to 50- fold greater than normal

hematopoietic system

increased T cell proliferation ( J:29355 )

• basal proliferation of T cells is 2.6 fold higher than controls

abnormal thymus lobule morphology ( J:29355 )

• leukemia leads to the loss of the bi-lobed structure of the thymus in mice over 3 months of age

thymus cortex hypoplasia ( J:29355 )

• leukemia in older mice leads to complete effacement of cortical and medullary structures in the thymus

abnormal thymus medulla morphology ( J:29355 )

• leukemia in older mice leads to complete effacement of cortical and medullary structures in the thymus

enlarged thymus ( J:29355 )

• leukemia leads to a swelling of the thymus in mice over 3 months of age

abnormal thymocyte activation ( J:29355 )

• thymocytes from 1 month old mice have a 200-fold increase in their proliferation rate in response to TCR stimulus compared to controls

• a less dramatic increase in proliferation rate is observed with thymocytes from E18.5 fetuses

spleen hyperplasia ( J:29355 )

• splenocyte numbers are 2- to 5- fold greater than controls by 3 months of age

• leukemia later in life leads to spleen cellularity that is 10-fold greater than normal

abnormal T cell differentiation ( J:29355 )

• starting at ages 2-3 months, intermediate single positive thymoyctes start accumulating

decreased spleen red pulp amount ( J:29355 )

• leukemia leads to an decrease in the red pulp area of the spleen

increased spleen white pulp amount ( J:29355 )

• leukemia leads to an increase in the white pulp area of the spleen

endocrine/exocrine glands

abnormal thymus lobule morphology ( J:29355 )

• leukemia leads to the loss of the bi-lobed structure of the thymus in mice over 3 months of age

thymus cortex hypoplasia ( J:29355 )

• leukemia in older mice leads to complete effacement of cortical and medullary structures in the thymus

abnormal thymus medulla morphology ( J:29355 )

• leukemia in older mice leads to complete effacement of cortical and medullary structures in the thymus

enlarged thymus ( J:29355 )

• leukemia leads to a swelling of the thymus in mice over 3 months of age

abnormal thymocyte activation ( J:29355 )

• thymocytes from 1 month old mice have a 200-fold increase in their proliferation rate in response to TCR stimulus compared to controls

• a less dramatic increase in proliferation rate is observed with thymocytes from E18.5 fetuses

cellular

increased T cell proliferation ( J:29355 )

• basal proliferation of T cells is 2.6 fold higher than controls

growth/size/body

spleen hyperplasia ( J:29355 )

• splenocyte numbers are 2- to 5- fold greater than controls by 3 months of age

• leukemia later in life leads to spleen cellularity that is 10-fold greater than normal