All Phenotypes Ikbkg<tm1.1Mpa> MGI Mouse

Phenotypes associated with this allele

Allelic Composition	Cyld^tm1.1Mpa/Cyld^tm1.1Mpa Ikbkg^tm1.1Mpa/Ikbkg^tm1.1Mpa Tg(Vil1-cre)997Gum/0
Genetic Background	B6.Cg-Cyld^tm1.1Mpa Ikbkg^tm1.1Mpa Tg(Vil1-cre)997Gum

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Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cyld^tm1.1Mpa mutation (0 available); any Cyld mutation (44 available)
Ikbkg^tm1.1Mpa 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

crypts of Lieberkuhn abscesses ( J:175865 )

colitis ( J:175865 )

• as in Ikbkg^tm1.1Mpa/Ikbkg^tm1.1Mpa Tg(Vil-cre)997Gum

endocrine/exocrine glands

crypts of Lieberkuhn abscesses ( J:175865 )

immune system

colitis ( J:175865 )

• as in Ikbkg^tm1.1Mpa/Ikbkg^tm1.1Mpa Tg(Vil-cre)997Gum

Allelic Composition	Cyld^tm1.1Mpa/Cyld^tm1.1Mpa Ikbkg^tm1.1Mpa/Y Tg(Vil1-cre)997Gum/0
Genetic Background	B6.Cg-Cyld^tm1.1Mpa Ikbkg^tm1.1Mpa Tg(Vil1-cre)997Gum

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

digestive/alimentary system

crypts of Lieberkuhn abscesses ( J:175865 )

colitis ( J:175865 )

• as in Ikbkg^tm1.1Mpa/Ikbkg^tm1.1Mpa Tg(Vil-cre)997Gum

endocrine/exocrine glands

crypts of Lieberkuhn abscesses ( J:175865 )

immune system

colitis ( J:175865 )

• as in Ikbkg^tm1.1Mpa/Ikbkg^tm1.1Mpa Tg(Vil-cre)997Gum

Allelic Composition	Ikbkg^tm1.1Mpa/Ikbkg^tm1.1Mpa Tg(Vil1-cre)997Gum/0
Genetic Background	B6.Cg-Ikbkg^tm1.1Mpa Tg(Vil1-cre)997Gum

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ikbkg^tm1.1Mpa 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

crypts of Lieberkuhn abscesses ( J:175865 )

colitis ( J:175865 )

• severe and chronic

endocrine/exocrine glands

crypts of Lieberkuhn abscesses ( J:175865 )

immune system

colitis ( J:175865 )

• severe and chronic

Allelic Composition	Ikbkg^tm1.1Mpa/Y Tg(Vil1-cre)997Gum/0
Genetic Background	B6.Cg-Ikbkg^tm1.1Mpa Tg(Vil1-cre)997Gum

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ikbkg^tm1.1Mpa 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

crypts of Lieberkuhn abscesses ( J:175865 )

colitis ( J:175865 )

• severe and chronic

endocrine/exocrine glands

crypts of Lieberkuhn abscesses ( J:175865 )

immune system

colitis ( J:175865 )

• severe and chronic

Allelic Composition	Cd19^tm1(cre)Cgn/Cd19⁺ Ikbkg^tm1.1Mpa/Y
Genetic Background	involves: 129P2/OlaHsd * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cd19^tm1(cre)Cgn mutation (11 available); any Cd19 mutation (60 available)
Ikbkg^tm1.1Mpa mutation (1 available); any Ikbkg mutation (16 available)

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

immune system

increased B cell proliferation ( J:79132 )

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

decreased immature B cell number ( J:79132 )

• immature B cell numbers in the spleen are reduced by about a third

decreased mature B cell number ( J:79132 )

• there is a 2- to 4- fold reduction in the number of mature recirculating B cells found in the bone marrow

• the IgM^lowIgD⁺ 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

decreased B-1 B cell number ( J:79132 )

• B-1 B cell numbers are strongly decreased in the peritoneum cavity

decreased follicular B cell number ( J:79132 )

• follicular B cells are the most diminished B cell population in the spleen

decreased marginal zone B cell number ( J:79132 )

• marginal zone B cell numbers are reduced about 4-fold

hematopoietic system

increased B cell proliferation ( J:79132 )

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

decreased immature B cell number ( J:79132 )

• immature B cell numbers in the spleen are reduced by about a third

decreased mature B cell number ( J:79132 )

• there is a 2- to 4- fold reduction in the number of mature recirculating B cells found in the bone marrow

• the IgM^lowIgD⁺ 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

decreased B-1 B cell number ( J:79132 )

• B-1 B cell numbers are strongly decreased in the peritoneum cavity

decreased follicular B cell number ( J:79132 )

• follicular B cells are the most diminished B cell population in the spleen

decreased marginal zone B cell number ( J:79132 )

• marginal zone B cell numbers are reduced about 4-fold

cellular

increased B cell proliferation ( J:79132 )

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

Allelic Composition	Ikbkg^tm1.1Mpa/Ikbkg^tm1.1Mpa Cd79a^tm1(cre)Reth/Cd79a⁺
Genetic Background	involves: BALB/c * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cd79a^tm1(cre)Reth mutation (3 available); any Cd79a mutation (24 available)
Ikbkg^tm1.1Mpa mutation (1 available); any Ikbkg mutation (16 available)

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

immune system

abnormal B cell differentiation ( J:113365 )

• there is a pronounced block in B cell development at transition stage 1 in the spleen

decreased B cell number ( J:113365 )

• mice show dramatic reduction in peripheral B cell numbers

decreased mature B cell number ( J:113365 )

• numbers of mature B cells are reduced to 10% of levels in controls

hematopoietic system

abnormal B cell differentiation ( J:113365 )

• there is a pronounced block in B cell development at transition stage 1 in the spleen

decreased B cell number ( J:113365 )

• mice show dramatic reduction in peripheral B cell numbers

decreased mature B cell number ( J:113365 )

• numbers of mature B cells are reduced to 10% of levels in controls

Allelic Composition	Ikbkg^tm1.1Mpa/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:
Ikbkg^tm1.1Mpa 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

increased T cell apoptosis ( J:85810 )

• increase in the numbers of apoptotic CD8+/HAS^low/- and CD4+/HAS^low/- mature thymocytes

decreased T cell number ( J:85810 )

• spleen and lymph nodes are nearly devoid of T cells

decreased CD4-positive, alpha-beta T cell number ( J:85810 )

• only a mild reduction in the CD4 single-positive cells in the thymus

decreased CD8-positive, alpha-beta T cell number ( J:85810 )

• 50% reduction in CD8 single-positive cells in the thymus

decreased single-positive T cell number ( J:85810 )

• loss of single-positive thymocytes during final maturation stages in the thymus

hematopoietic system

increased T cell apoptosis ( J:85810 )

• increase in the numbers of apoptotic CD8+/HAS^low/- and CD4+/HAS^low/- mature thymocytes

decreased T cell number ( J:85810 )

• spleen and lymph nodes are nearly devoid of T cells

decreased CD4-positive, alpha-beta T cell number ( J:85810 )

• only a mild reduction in the CD4 single-positive cells in the thymus

decreased CD8-positive, alpha-beta T cell number ( J:85810 )

• 50% reduction in CD8 single-positive cells in the thymus

decreased single-positive T cell number ( J:85810 )

• loss of single-positive thymocytes during final maturation stages in the thymus

cellular

increased T cell apoptosis ( J:85810 )

• increase in the numbers of apoptotic CD8+/HAS^low/- and CD4+/HAS^low/- mature thymocytes

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

liver/biliary system

liver inflammation ( J:118336 )

• 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

abnormal liver morphology ( J:118336 )

• at 6 months, livers show macroscopic nodules

• focal areas of lipid accumulation and low-grade dysplastic nodules with expansive growth are observed

abnormal hepatocyte morphology ( J:118336 )

• 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

abnormal hepatocyte mitochondrial morphology ( J:118336 )

• mitochondria of hepatocytes appear swollen and display a reduction in mitochondrial crests compared to wild-type at 8 weeks of age

hepatic steatosis ( J:118336 )

• at 6 months, mice show signs of steatosis and increased lipid deposition in hepatocytes

increased hepatocellular carcinoma incidence ( J:118336 )

• 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

increased hepatocellular carcinoma incidence ( J:118336 )

• 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 increased proliferation and higher mitotic indices (~1.44 per high-power field)

• tumors at 9 months show dysplastic foci and nodules

immune system

abnormal cytokine secretion ( J:118336 )

• cytokine expression is upregulated in mutants

abnormal inflammatory response ( J:118336 )

• upon LPS injection, NF kappa b activation is completely absent

increased susceptibility to endotoxin shock ( J:137861 )

• following exposure to LPS, mice exhibit increased liver failure and hepatocyte apoptosis compared to similarly treated wild-type mice

liver inflammation ( J:118336 )

• 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

abnormal hepatocyte mitochondrial morphology ( J:118336 )

• mitochondria of hepatocytes appear swollen and display a reduction in mitochondrial crests compared to wild-type at 8 weeks of age

increased apoptosis ( J:118336 )

• 8-week old mice show an increase in apoptotic cells in the liver

increased cell proliferation ( J:118336 )

• 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

decreased circulating glucose level ( J:118336 )

• mice show a decrease in spontaneous serum glucose levels compared to controls

increased circulating alanine transaminase level ( J:118336 )

• mice show elevated serum levels of alanine aminotransferase (ALT) at 3 weeks, indicative of hepatocyte damage; ALT levels are further increased at 8 weeks

abnormal enzyme/coenzyme activity ( J:118336 )

• 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

Allelic Composition	Fadd^tm1Mpa/Fadd^tm1Mpa Ikbkg^tm1.1Mpa/Ikbkg^tm1.1Mpa Tg(Alb1-cre)7Gsc/0
Genetic Background	involves: C57BL/6 * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fadd^tm1Mpa mutation (0 available); any Fadd mutation (18 available)
Ikbkg^tm1.1Mpa 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

decreased circulating alanine transaminase level ( J:118336 )

• spontaneous serum ALT levels are decreased compared to single Ikbkg mutants at 8 weeks of age

abnormal enzyme/coenzyme activity ( J:118336 )

• mice show increased JNK kinase activity

immune system

liver inflammation ( J:118336 )

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

liver/biliary system

liver/biliary system phenotype ( J:118336 )

N	• liver inflammation, hepatocyte apoptosis, increased hepatocyte proliferation, and focal lipid accumulation are inhibited in mutants compared to Ikbkg single mutants

liver inflammation ( J:118336 )

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

cellular

decreased apoptosis ( J:118336 )

• double mutants are protected from LPS-induced hepatocyte apoptosis

Contributing Projects: 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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