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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Ikbkgtm1.1Mpa
targeted mutation 1.1, Manolis Pasparakis
MGI:2679024
Summary 9 genotypes


Genotype
MGI:5293401
cn1
Allelic
Composition
Cyldtm1.1Mpa/Cyldtm1.1Mpa
Ikbkgtm1.1Mpa/Ikbkgtm1.1Mpa
Tg(Vil1-cre)997Gum/0
Genetic
Background
B6.Cg-Cyldtm1.1Mpa Ikbkgtm1.1Mpa Tg(Vil1-cre)997Gum
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cyldtm1.1Mpa mutation (0 available); any Cyld mutation (44 available)
Ikbkgtm1.1Mpa mutation (1 available); any Ikbkg mutation (16 available)
Tg(Vil1-cre)997Gum mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
digestive/alimentary system
• as in Ikbkgtm1.1Mpa/Ikbkgtm1.1Mpa Tg(Vil-cre)997Gum

endocrine/exocrine glands

immune system
• as in Ikbkgtm1.1Mpa/Ikbkgtm1.1Mpa Tg(Vil-cre)997Gum




Genotype
MGI:5293402
cn2
Allelic
Composition
Cyldtm1.1Mpa/Cyldtm1.1Mpa
Ikbkgtm1.1Mpa/Y
Tg(Vil1-cre)997Gum/0
Genetic
Background
B6.Cg-Cyldtm1.1Mpa Ikbkgtm1.1Mpa Tg(Vil1-cre)997Gum
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cyldtm1.1Mpa mutation (0 available); any Cyld mutation (44 available)
Ikbkgtm1.1Mpa mutation (1 available); any Ikbkg mutation (16 available)
Tg(Vil1-cre)997Gum mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
digestive/alimentary system
• as in Ikbkgtm1.1Mpa/Ikbkgtm1.1Mpa Tg(Vil-cre)997Gum

endocrine/exocrine glands

immune system
• as in Ikbkgtm1.1Mpa/Ikbkgtm1.1Mpa Tg(Vil-cre)997Gum




Genotype
MGI:5293403
cn3
Allelic
Composition
Ikbkgtm1.1Mpa/Ikbkgtm1.1Mpa
Tg(Vil1-cre)997Gum/0
Genetic
Background
B6.Cg-Ikbkgtm1.1Mpa Tg(Vil1-cre)997Gum
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ikbkgtm1.1Mpa mutation (1 available); any Ikbkg mutation (16 available)
Tg(Vil1-cre)997Gum mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
digestive/alimentary system
• severe and chronic

endocrine/exocrine glands

immune system
• severe and chronic




Genotype
MGI:5293406
cn4
Allelic
Composition
Ikbkgtm1.1Mpa/Y
Tg(Vil1-cre)997Gum/0
Genetic
Background
B6.Cg-Ikbkgtm1.1Mpa Tg(Vil1-cre)997Gum
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ikbkgtm1.1Mpa mutation (1 available); any Ikbkg mutation (16 available)
Tg(Vil1-cre)997Gum mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
digestive/alimentary system
• severe and chronic

endocrine/exocrine glands

immune system
• severe and chronic




Genotype
MGI:3851695
cn5
Allelic
Composition
Cd19tm1(cre)Cgn/Cd19+
Ikbkgtm1.1Mpa/Y
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cd19tm1(cre)Cgn mutation (11 available); any Cd19 mutation (60 available)
Ikbkgtm1.1Mpa mutation (1 available); any Ikbkg mutation (16 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
immune system
• B cell turnover over a one week period is twice that of controls
• immature B cell numbers in the spleen are reduced by about a third
• there is a 2- to 4- fold reduction in the number of mature recirculating B cells found in the bone marrow
• the IgMlowIgD+ mature B cell population is strongly diminished in the spleen by 3- to 4- fold
• B cell numbers in the lymph nodes are also strongly reduced
• B-1 B cell numbers are strongly decreased in the peritoneum cavity
• follicular B cells are the most diminished B cell population in the spleen
• marginal zone B cell numbers are reduced about 4-fold

hematopoietic system
• B cell turnover over a one week period is twice that of controls
• immature B cell numbers in the spleen are reduced by about a third
• there is a 2- to 4- fold reduction in the number of mature recirculating B cells found in the bone marrow
• the IgMlowIgD+ mature B cell population is strongly diminished in the spleen by 3- to 4- fold
• B cell numbers in the lymph nodes are also strongly reduced
• B-1 B cell numbers are strongly decreased in the peritoneum cavity
• follicular B cells are the most diminished B cell population in the spleen
• marginal zone B cell numbers are reduced about 4-fold

cellular
• B cell turnover over a one week period is twice that of controls




Genotype
MGI:3687508
cn6
Allelic
Composition
Ikbkgtm1.1Mpa/Ikbkgtm1.1Mpa
Cd79atm1(cre)Reth/Cd79a+
Genetic
Background
involves: BALB/c * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cd79atm1(cre)Reth mutation (3 available); any Cd79a mutation (24 available)
Ikbkgtm1.1Mpa mutation (1 available); any Ikbkg mutation (16 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
immune system
• there is a pronounced block in B cell development at transition stage 1 in the spleen
• mice show dramatic reduction in peripheral B cell numbers
• numbers of mature B cells are reduced to 10% of levels in controls

hematopoietic system
• there is a pronounced block in B cell development at transition stage 1 in the spleen
• mice show dramatic reduction in peripheral B cell numbers
• numbers of mature B cells are reduced to 10% of levels in controls




Genotype
MGI:2679026
cn7
Allelic
Composition
Ikbkgtm1.1Mpa/Y
Tg(Cd4-cre)1Cwi/0
Genetic
Background
involves: C57BL/6 * DBA/2
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ikbkgtm1.1Mpa mutation (1 available); any Ikbkg mutation (16 available)
Tg(Cd4-cre)1Cwi mutation (10 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
immune system
• increase in the numbers of apoptotic CD8+/HASlow/- and CD4+/HASlow/- mature thymocytes
• spleen and lymph nodes are nearly devoid of T cells
• only a mild reduction in the CD4 single-positive cells in the thymus
• 50% reduction in CD8 single-positive cells in the thymus
• loss of single-positive thymocytes during final maturation stages in the thymus

hematopoietic system
• increase in the numbers of apoptotic CD8+/HASlow/- and CD4+/HASlow/- mature thymocytes
• spleen and lymph nodes are nearly devoid of T cells
• only a mild reduction in the CD4 single-positive cells in the thymus
• 50% reduction in CD8 single-positive cells in the thymus
• loss of single-positive thymocytes during final maturation stages in the thymus

cellular
• increase in the numbers of apoptotic CD8+/HASlow/- and CD4+/HASlow/- mature thymocytes




Genotype
MGI:3700375
cn8
Allelic
Composition
Ikbkgtm1.1Mpa/Ikbkgtm1.1Mpa
Tg(Alb1-cre)7Gsc/0
Genetic
Background
involves: C57BL/6 * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ikbkgtm1.1Mpa mutation (1 available); any Ikbkg mutation (16 available)
Tg(Alb1-cre)7Gsc mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
liver/biliary system
• 8 week old mice show signs of steatohepatitis in the liver, characterized by immune cell infiltration into the parenchyma
• in 8-week old mice, hepatocytes show features of large-cell dysplasia with strong anisokaryosis; large areas of irregularly shaped glycogen deposits are detected
• at 6 months, livers show macroscopic nodules
• focal areas of lipid accumulation and low-grade dysplastic nodules with expansive growth are observed
• at 6 months, hepatocytes show lipid deposition and ballooning
• in 8-week old mice, hepatocytes show features of large-cell dysplasia with strong anisokaryosis; large areas of irregularly shaped glycogen deposits are detected
• mitochondria of hepatocytes appear swollen and display a reduction in mitochondrial crests compared to wild-type at 8 weeks of age
• at 6 months, mice show signs of steatosis and increased lipid deposition in hepatocytes
• multiple tumors are found in livers of 12-month old males; malignant liver tumors are present in all mice examined
• average number of tumors per mouse is 5.75 with average size of 8.58 mm
• tumors show expansive growth, increased cellularity, broad trabecular growth pattern in 4 to 6 layers, eosinophilia, higher cytoplasmic index compared to non malignant areas, and complete absence of portal tracts
• tumors show increased proliferation and higher mitotic indices (~1.44 per high-power field)
• tumors at 9 months show dysplastic foci and nodules

neoplasm
• multiple tumors are found in livers of 12-month old males; malignant liver tumors are present in all mice examined
• average number of tumors per mouse is 5.75 with average size of 8.58 mm
• tumors show expansive growth, increased cellularity, broad trabecular growth pattern in 4 to 6 layers, eosinophilia, higher cytoplasmic index compared to non malignant areas, and complete absence of portal tracts
• tumors show increased proliferation and higher mitotic indices (~1.44 per high-power field)
• tumors at 9 months show dysplastic foci and nodules

immune system
• cytokine expression is upregulated in mutants
• upon LPS injection, NF kappa b activation is completely absent
• following exposure to LPS, mice exhibit increased liver failure and hepatocyte apoptosis compared to similarly treated wild-type mice
• 8 week old mice show signs of steatohepatitis in the liver, characterized by immune cell infiltration into the parenchyma
• in 8-week old mice, hepatocytes show features of large-cell dysplasia with strong anisokaryosis; large areas of irregularly shaped glycogen deposits are detected

cellular
• mitochondria of hepatocytes appear swollen and display a reduction in mitochondrial crests compared to wild-type at 8 weeks of age
• 8-week old mice show an increase in apoptotic cells in the liver
• livers show increased proliferation
• at 8 weeks, there is a significant increase in progenitor cells and activation of oval cells in the liver

homeostasis/metabolism
• mice show a decrease in spontaneous serum glucose levels compared to controls
• mice show elevated serum levels of alanine aminotransferase (ALT) at 3 weeks, indicative of hepatocyte damage; ALT levels are further increased at 8 weeks
• mice show increased JNK kinase activity

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
steatotic liver disease DOID:9452 OMIM:228100
J:118336




Genotype
MGI:3700376
cn9
Allelic
Composition
Faddtm1Mpa/Faddtm1Mpa
Ikbkgtm1.1Mpa/Ikbkgtm1.1Mpa
Tg(Alb1-cre)7Gsc/0
Genetic
Background
involves: C57BL/6 * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Faddtm1Mpa mutation (0 available); any Fadd mutation (18 available)
Ikbkgtm1.1Mpa mutation (1 available); any Ikbkg mutation (16 available)
Tg(Alb1-cre)7Gsc mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
homeostasis/metabolism
• spontaneous serum ALT levels are decreased compared to single Ikbkg mutants at 8 weeks of age
• mice show increased JNK kinase activity

immune system
• completely absent compared to single Ikbkg mutants at 8 weeks of age

liver/biliary system
N
• liver inflammation, hepatocyte apoptosis, increased hepatocyte proliferation, and focal lipid accumulation are inhibited in mutants compared to Ikbkg single mutants
• completely absent compared to single Ikbkg mutants at 8 weeks of age

cellular
• double mutants are protected from LPS-induced hepatocyte apoptosis





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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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last database update
11/12/2024
MGI 6.24
The Jackson Laboratory