Description

The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.

Strain Information

Type Mutant Stock; Targeted Mutation; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Species laboratory mouse Generation ?+N1pN1 Generation Definitions   Donating Investigator David Baltimore,   California Institute of Technology

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.

Control Information

	Control
	Wild-type from the colony
	101045 B6129SF2/J	(approximate)
Considerations for Choosing Controls

Additional Web Information

JAX^® NOTES, Summer 2005; 498. Toll-like Receptor JAX^® Mice for Immunological Research.

Phenotype

Phenotype Information

View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI

      assigned by genotype

Nfkbia^tm1Bal/Nfkbia^tm1Bal

        involves: 129S4/SvJae * C57BL/6J

• mortality/aging
• complete postnatal lethality
  • death occurred around the 1st week of age   (MGI Ref ID J:30058)
• growth/size phenotype
• decreased body size
  • although of normal size at birth, homozygotes became noticeably smaller than normal by 3-4 days of age   (MGI Ref ID J:30058)
  • by 7 days of age homozygotes were severely runted (1/3 normal weight)   (MGI Ref ID J:30058)
• hematopoietic system phenotype
• abnormal granulocyte differentiation
  • elevated numbers of granulocyte precursors in bone marrow and spleen as animals age   (MGI Ref ID J:30058)
• decreased double-positive T cell number
  • after 6 days   (MGI Ref ID J:30058)
• increased leukocyte cell number
  • 3 fold increase in peripheral WBC count - about 95% neutrophils   (MGI Ref ID J:30058)
• spleen atrophy
  • small size by day 2 with a worsening size reduction until death   (MGI Ref ID J:30058)
• thymus atrophy
  • atrophy in mice over 6 days of age   (MGI Ref ID J:30058)
• immune system phenotype
• abnormal granulocyte differentiation
  • elevated numbers of granulocyte precursors in bone marrow and spleen as animals age   (MGI Ref ID J:30058)
• decreased double-positive T cell number
  • after 6 days   (MGI Ref ID J:30058)
• increased leukocyte cell number
  • 3 fold increase in peripheral WBC count - about 95% neutrophils   (MGI Ref ID J:30058)
• spleen atrophy
  • small size by day 2 with a worsening size reduction until death   (MGI Ref ID J:30058)
• thymus atrophy
  • atrophy in mice over 6 days of age   (MGI Ref ID J:30058)
• integument phenotype
• abnormal epidermis stratum basale morphology
  • poorly defined basal layer   (MGI Ref ID J:30058)
• abnormal skin condition   (MGI Ref ID J:30058)
• flaky skin
  • significant sloughing   (MGI Ref ID J:30058)
• scaly skin   (MGI Ref ID J:30058)
• cellular phenotype
• abnormal granulocyte differentiation
  • elevated numbers of granulocyte precursors in bone marrow and spleen as animals age   (MGI Ref ID J:30058)
• decreased double-positive T cell number
  • after 6 days   (MGI Ref ID J:30058)

View Research Applications

Research Applications

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

Nfkbia^tm1Bal related

Dermatology Research
Skin and Hair Texture Defects

Developmental Biology Research
Growth Defects

Immunology and Inflammation Research
Immunodeficiency

Genes & Alleles

Gene & Allele Information provided by MGI

Allele Symbol		Nfkbiatm1Bal
Allele Name		targeted mutation 1, David Baltimore
Allele Type		Targeted (Reporter)
Common Name(s)		IkappaBalpha-;
Mutation Made By		David Baltimore,   California Institute of Technology
Strain of Origin		129S4/SvJae
ES Cell Line Name		J1
ES Cell Line Strain		129S4/SvJae
Gene Symbol and Name		Nfkbia, nuclear factor of kappa light polypeptide gene enhancer in B cells inhibitor, alpha
Chromosome		12
Gene Common Name(s)		AI462015; I(Kappa)B(alpha); I-kappaBalpha; IKBA; IkappaBalpha; MAD-3; NFKBI; Nfkbi; RL/IF-1; expressed sequence AI462015; nuclear factor of kappa light chain gene enhancer in B-cells inhibitor;
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. [MGI Ref ID J:30058]

Genotyping

Genotyping Information

Genotyping Protocols

NEOTD (Generic Neo), Standard PCR

Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

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):2736-46. [PubMed: 7590249]  [MGI Ref ID J:30058]

Additional References

Nfkbia^tm1Bal related

Delhase M; Kim SY; Lee H; Naiki-Ito A; Chen Y; Ahn ER; Murata K; Kim SJ; Lautsch N; Kobayashi KS; Shirai T; Karin M; Nakanishi M. 2011. TANK-binding kinase 1 (TBK1) controls cell survival through PAI-2/serpinB2 and transglutaminase 2. Proc Natl Acad Sci U S A :. [PubMed: 22203995]  [MGI Ref ID J:179973]

Goudeau B; Huetz F; Samson S; Di Santo JP; Cumano A; Beg A; Israel A; Memet S. 2003. IkappaBalpha/IkappaBepsilon deficiency reveals that a critical NF-kappaB dosage is required for lymphocyte survival. Proc Natl Acad Sci U S A 100(26):15800-5. [PubMed: 14665694]  [MGI Ref ID J:88142]

Groesdonk HV; Wagner F; Hoffarth B; Georgieff M; Senftleben U. 2007. Enhancement of NF-kappaB activation in lymphocytes prevents T cell apoptosis and improves survival in murine sepsis. J Immunol 179(12):8083-9. [PubMed: 18056349]  [MGI Ref ID J:155202]

Samson SI; Memet S; Vosshenrich CA; Colucci F; Richard O; Ndiaye D; Israel A; Di Santo JP. 2004. Combined deficiency in IkappaBalpha and IkappaBepsilon reveals a critical window of NF-kappaB activity in natural killer cell differentiation. Blood 103(12):4573-80. [PubMed: 14764534]  [MGI Ref ID J:90970]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, RG10/RG30.

Purchasing information

Pricing, Supply Level & Notes, Controls

General Supply Notes

• This strain is included in the Induced Mutant Resource Colony collection.
• Genomic DNA is available for this strain from the Mouse DNA Resource.

Control Information

	Control
	Wild-type from the colony
	101045 B6129SF2/J	(approximate)
Considerations for Choosing Controls
Control Pricing Information for Genetically Engineered Mutant Strains.

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The Jackson Laboratory's Genotype Promise

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.

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