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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Nfkb1tm1Brv
targeted mutation 1, Rodrigo Bravo
MGI:2179707
Summary 4 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
hm1
Nfkb1tm1Brv/Nfkb1tm1Brv involves: 129S1/Sv MGI:3768242
hm2
Nfkb1tm1Brv/Nfkb1tm1Brv involves: 129S1/Sv * C57BL/6 MGI:3852530
cx3
Nfkb1tm1Brv/Nfkb1tm1Brv
Nfkb2tm1Brv/Nfkb2tm1Brv
involves: 129S1/Sv * C57BL/6 MGI:3852529
cx4
Nfkb1tm1Brv/Nfkb1tm1Brv
Nfkb2tm1Brv/Nfkb2+
involves: 129S1/Sv * C57BL/6 MGI:3852531


Genotype
MGI:3768242
hm1
Allelic
Composition
Nfkb1tm1Brv/Nfkb1tm1Brv
Genetic
Background
involves: 129S1/Sv
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Nfkb1tm1Brv mutation (0 available); any Nfkb1 mutation (92 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cellular
• at low levels of stimulation, B cell proliferation is increased 4- to 5-foldcompared to in wild-type mice
• T cell proliferation stimulated by anti-CD3 or anti-CD3 and anti-CD28 antibodies do not proliferate as well as wild-type T cells
• however, proliferation stimulated by PMA and PHA is normal

mortality/aging
• mice begin to die after 2 months and survival at 100 days is 50% whereas almost all wild-type mice are alive at day 100
• 20% of mice die prior to weaning

immune system
• mice exhibit myeloid hyperplasia in the bone marrow associated with decreased erythrocyte cell number
• single positive CD4+ or CD8+ T cells are decreased in the spleen and lymph node
• the number of Thy 1.2+ cells is decreased by 50% compared to wild-type in the peripheral T cell population
• at low levels of stimulation, B cell proliferation is increased 4- to 5-foldcompared to in wild-type mice
• T cell proliferation stimulated by anti-CD3 or anti-CD3 and anti-CD28 antibodies do not proliferate as well as wild-type T cells
• however, proliferation stimulated by PMA and PHA is normal
• IgG3 levels are decreased 4-fold
• serum Ig levels are increased 5-fold
• IgE levels are increased 50-fold
• IgG1 levels are increased 18-fold
• mice develop spleen enlargement due to extramedullary hematopoiesis and follicular expansion with loss of mantle and marginal zone differentiation at 3 weeks of age that worsens with age
• mice develop lymph node enlargement at 3 weeks that worsens with age
• however, lymphoid cell numbers are not increased
• after 2 and 3 weeks stimulated with NP-KLH, IgG1 production is increased 2- to 3-fold compared to in wild-type mice
• after 3 weeks of stimulation with LPS, IgG3 levels are reduced 3-fold
• however, basal and specific antibody production is not seriously affected
• stimulated and non-stimulated macrophages produce less cytokines that wild-type cells (17- to 86-fold reduction in unstimulated cells and 2- to 8-fold reduction in stimulated cells)
• stimulated T cells produce 3- to 6-fold less IL-2 than wild-type
• stimulated T cells produce 3- to 6-fold less IL-4 than wild-type
• stimulated T cells produce 3- to 6-fold less TNF-alpha than wild-type
• mild to moderate perivascular and periportal mixed infiltration of inflammatory cells is mainly composed of lymphocytic cells
• mild to moderate perivascular and periairway mixed infiltration of inflammatory cells is mainly composed of lymphocytic cells
• mice in conventional housing develop eye and/or nasal infections produced by Staphylococcus xylosis and Pasteurella haemolytica unlike wild-type mice
• however, mice housed in microinsolators do not develop infections

growth/size/body
• after weaning mice exhibit growth retardation

behavior/neurological
• after weaning surviving mice appear sick, have rough coats and are lethargic

hematopoietic system
• mice exhibit myeloid hyperplasia in the bone marrow associated with decreased erythrocyte cell number
• extramedullary hematopoiesis results in splenomegaly at 3 weeks and worsens with age
• erythroblasts accumulate in the spleen
• erythroblasts are decreased in the bone marrow whereas granulocytes are slightly increased
• decreased erythrocyte cell numbers are associated with myeloid hyperplasia in the bone marrow
• single positive CD4+ or CD8+ T cells are decreased in the spleen and lymph node
• the number of Thy 1.2+ cells is decreased by 50% compared to wild-type in the peripheral T cell population
• mice develop spleen enlargement due to extramedullary hematopoiesis and follicular expansion with loss of mantle and marginal zone differentiation at 3 weeks of age that worsens with age
• at low levels of stimulation, B cell proliferation is increased 4- to 5-foldcompared to in wild-type mice
• T cell proliferation stimulated by anti-CD3 or anti-CD3 and anti-CD28 antibodies do not proliferate as well as wild-type T cells
• however, proliferation stimulated by PMA and PHA is normal
• IgG3 levels are decreased 4-fold
• serum Ig levels are increased 5-fold
• IgE levels are increased 50-fold
• IgG1 levels are increased 18-fold

liver/biliary system
• mild to moderate perivascular and periportal mixed infiltration of inflammatory cells is mainly composed of lymphocytic cells

respiratory system
• mild to moderate perivascular and periairway mixed infiltration of inflammatory cells is mainly composed of lymphocytic cells

integument
• after weaning surviving mice appear sick, have rough coats and are lethargic




Genotype
MGI:3852530
hm2
Allelic
Composition
Nfkb1tm1Brv/Nfkb1tm1Brv
Genetic
Background
involves: 129S1/Sv * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Nfkb1tm1Brv mutation (0 available); any Nfkb1 mutation (92 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
skeleton
N
• mice exhibit normal bone structure

immune system

hematopoietic system




Genotype
MGI:3852529
cx3
Allelic
Composition
Nfkb1tm1Brv/Nfkb1tm1Brv
Nfkb2tm1Brv/Nfkb2tm1Brv
Genetic
Background
involves: 129S1/Sv * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Nfkb1tm1Brv mutation (0 available); any Nfkb1 mutation (92 available)
Nfkb2tm1Brv mutation (0 available); any Nfkb2 mutation (38 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
skeleton
• fewer osteoclasts form while more bone-residing macrophages are detected compared to in wild-type mice
• tooth eruption is prevented by a superficial bone plate on the mandible and maxilla and impacted teeth are poorly invested in the surrounding alveolar bone unlike in wild-type mice
• however, mice reconstituted with wild-type marrow exhibit only delayed tooth eruption
• persistent cartilaginous and osseous trabecular are found throughout the epiphysis, metaphysis and diaphysis unlike in wild-type mice
• endochondral bones exhibit thin, hypocellular growth plates and wide, cavernous marrow spaces unlike in wild-type mice
• the proliferating and hypertrophic zones are arranged in irregular clusters unlike the discrete columns observed in wild-type mice
• the bone marrow cavity is filled with unresorbed primary spongiosa that reduces the space 80% compared to in wild-type mice

immune system
• fewer osteoclasts form while more bone-residing macrophages are detected compared to in wild-type mice
• the number of bone-residing macrophages is increased compared to in wild-type mice
• the number of macrophages in the spleen, blood, and bone marrow is increased compared to in wild-type mice
• LPS and IFN-gamma-stimulated macrophages exhibit reduced IL6 and GM-CSF (granulocyte monocyte colony stimulating factor) production compared to similarly treated wild-type cell
• LPS and IFN-gamma-stimulated macrophages exhibit reduced IL6 production compared to similarly treated wild-type cell

growth/size/body
• tooth eruption is prevented by a superficial bone plate on the mandible and maxilla and impacted teeth are poorly invested in the surrounding alveolar bone unlike in wild-type mice
• however, mice reconstituted with wild-type marrow exhibit only delayed tooth eruption

craniofacial
• tooth eruption is prevented by a superficial bone plate on the mandible and maxilla and impacted teeth are poorly invested in the surrounding alveolar bone unlike in wild-type mice
• however, mice reconstituted with wild-type marrow exhibit only delayed tooth eruption

hematopoietic system
• fewer osteoclasts form while more bone-residing macrophages are detected compared to in wild-type mice
• the number of bone-residing macrophages is increased compared to in wild-type mice
• the number of macrophages in the spleen, blood, and bone marrow is increased compared to in wild-type mice

cellular
• fewer osteoclasts form while more bone-residing macrophages are detected compared to in wild-type mice




Genotype
MGI:3852531
cx4
Allelic
Composition
Nfkb1tm1Brv/Nfkb1tm1Brv
Nfkb2tm1Brv/Nfkb2+
Genetic
Background
involves: 129S1/Sv * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Nfkb1tm1Brv mutation (0 available); any Nfkb1 mutation (92 available)
Nfkb2tm1Brv mutation (0 available); any Nfkb2 mutation (38 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
skeleton
• mice exhibit only subtle changes in bone structure
• osteoclast differentiation is arrested before fusion
• no mature osteoclasts are detected in cultures of spleen cells unlike those of wild-type cell

immune system
• osteoclast differentiation is arrested before fusion
• no mature osteoclasts are detected in cultures of spleen cells unlike those of wild-type cell

hematopoietic system
• osteoclast differentiation is arrested before fusion
• no mature osteoclasts are detected in cultures of spleen cells unlike those of wild-type cell

cellular
• osteoclast differentiation is arrested before fusion





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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last database update
09/10/2019
MGI 6.14
The Jackson Laboratory