Overview

Also Known As: IκBα-

Mice homozygous for the Nfkbia^tm1Bal mutation are runted, and have granulocytosis as well as abnormalities in epithelial keratinization. Neonates die at around 8 days of age.

Donating Investigator

Dr. David Baltimore, California Institute of Technology

Genetic overview

Genetic Background	Generation

Allele Type	Gene Symbol	Gene Name
Targeted (Reporter, Null/Knockout)	Nfkbia	nuclear factor of kappa light polypeptide gene enhancer in B cells inhibitor, alpha

Research Applications

Dermatology Research
Developmental Biology Research
Immunology, Inflammation and Autoimmunity Research

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Base Price

Starting at:

$2,854.50 Domestic price Cryo Recovery

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Details

Detailed Description

Mice homozygous for the Nfkbia^tm1Bal mutation are runted, and have granulocytosis as well as abnormalities in epithelial keratinization. Neonates die at around 8 days of age.

Development

The Nfkbia gene was disrupted using an in frame insertion vector containing lacZ. The 129-derived J1 ES cell line was used.

Expression Data

Expressed Gene	lacZ, beta-galactosidase, E. coli
Site of Expression

Control Suggestions

Wild-type from the colony

Approximate Controls

101045 B6129SF2/J

Additional Information

Considerations for Choosing Controls

Selected References

Beg AA; Sha WC; Bronson RT; Baltimore D. 1995. Constitutive NF-kappa B activation, enhanced granulopoiesis, and neonatal lethality in I kappa B alpha-deficient mice. Genes Dev 9(22):2736-46PubMed: 7590249 MGI: J:30058

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Genetics

Nfkbia^tm1Bal

Allele Symbol: Nfkbia^tm1Bal

Allele Name	targeted mutation 1, David Baltimore
Allele Type	Targeted (Reporter, Null/Knockout)
Allele Synonym(s)	IkappaBalpha^-
Gene Symbol and Name	Nfkbia, nuclear factor of kappa light polypeptide gene enhancer in B cells inhibitor, alpha
Gene Synonym(s)
Expressed Gene	lacZ, beta-galactosidase, E. coli
Strain of Origin	129S4/SvJae
Chromosome	12
Molecular Note	The lacZ gene was inserted in frame 13 nucleotides downstream of the translational start site followed by a PGK-neomycin gene in reverse orientation thus replacing the remainder of the gene. Absence of protein product was established by Western blot analysis.
Mutations Made By	Dr. David Baltimore, California Institute of Technology

Disease/Phenotype

Disease Terms

Research Areas By Phenotype

This mouse can be used to support research in many areas including:

Genotype: Nfkbia^tm1Bal related

Dermatology Research
- Skin and Hair Texture Defects
Developmental Biology Research
- Growth Defects
Immunology, Inflammation and Autoimmunity Research
- Immunodeficiency

Mammalian Phenotype Terms by Genotype

The following phenotype relates to a compound genotype created using this strain

Genotype: Nfkbia^tm1Bal/Nfkbia^tm1Bal
involves: 129S4/SvJae * C57BL/6J

cellular phenotype

abnormal granulocyte differentiation
- elevated numbers of granulocyte precursors in bone marrow and spleen as animals age

immune system phenotype

abnormal granulocyte differentiation
- elevated numbers of granulocyte precursors in bone marrow and spleen as animals age

decreased double-positive T cell number
- after 6 days

increased leukocyte cell number
- 3 fold increase in peripheral WBC count - about 95% neutrophils

spleen atrophy
- small size by day 2 with a worsening size reduction until death

thymus atrophy
- atrophy in mice over 6 days of age

integument phenotype

abnormal epidermis stratum basale morphology
- poorly defined basal layer

abnormal skin condition

flaky skin
- significant sloughing

scaly skin

mortality/aging

postnatal lethality, complete penetrance
- death occurred around the 1st week of age

endocrine/exocrine gland phenotype

thymus atrophy
- atrophy in mice over 6 days of age

growth/size/body region phenotype

decreased body size
- although of normal size at birth, homozygotes became noticeably smaller than normal by 3-4 days of age
- by 7 days of age homozygotes were severely runted (1/3 normal weight)

hematopoietic system phenotype

abnormal granulocyte differentiation
- elevated numbers of granulocyte precursors in bone marrow and spleen as animals age

decreased double-positive T cell number
- after 6 days

increased leukocyte cell number
- 3 fold increase in peripheral WBC count - about 95% neutrophils

spleen atrophy
- small size by day 2 with a worsening size reduction until death

thymus atrophy
- atrophy in mice over 6 days of age

References

Beg AA; Sha WC; Bronson RT; Baltimore D. 1995. Constitutive NF-kappa B activation, enhanced granulopoiesis, and neonatal lethality in I kappa B alpha-deficient mice. Genes Dev 9(22):2736-46PubMed: 7590249 MGI: J:30058

Additional - Nfkbia^tm1Bal related

Technical Support

CONTACT TECHNICAL SUPPORT

Genotyping Protocols
- Standard PCR:Nfkbiaalternate1
- Genotyping resources and troubleshooting
Breeding Considerations

Homozygous mice die ~8 days of age. When maintaining a live colony, heterozygous mice may be bred together or to wildtype mice from the colony.
- Additional Breeding and Husbandry Support
Citation
When using the IκBα- mouse strain in a publication, please cite the originating article(s) and include JAX stock #002850 in your Materials and Methods section.

Animal Health Reports

Facility Barrier Level Descriptions

Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, G200

Pricing & Availability

Cryo Recovery

Domestic
International

Live Mouse

Age

Genotype

Price

weeks

Cryorecovery - Pricing

Service/Product	Description	Price
	Heterozygous or Wild-type for Nfkbia<tm1Bal>

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The Jackson Laboratory has rigorous genetic quality control and mutant gene genotyping programs to ensure the genetic background of JAX® Mice strains as well as the genotypes of strains with identified molecular mutations. JAX® Mice strains are only made available to researchers after meeting our standards. However, the phenotype of each strain may not be fully characterized and/or captured in the strain data sheets. Therefore, we cannot guarantee a strain's phenotype will meet all expectations. To ensure that JAX® Mice will meet the needs of individual research projects or when requesting a strain that is new to your research, we suggest ordering and performing tests on a small number of mice to determine suitability for your particular project. We do not guarantee breeding performance and therefore suggest that investigators order more than one breeding pair to avoid delays in their research.

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