Strain Name:
B6J.Cg-Ikbkgtm2Dln Tnfrsf1atm1Mak/Mmucd
Stock Number:
041407-UCD
Citation ID:
RRID:MMRRC_041407-UCD
Other Names:
NEMO PtM HA, NH

Strain Information

Ikbkgtm2Dln
Name:
Type:
Species: Mus musculus (mouse)
Chromosome:
Ikbkg
Name: inhibitor of kappaB kinase gamma
Synonyms: 1110037D23Rik, NEMO, IKK[g]
Type: Gene
Species: Mus musculus (mouse)
Chromosome: X
NCBI: 16151
HGNC: HGNC:5961
Homologene: 2698
Tnfrsf1atm1Mak
Name: tumor necrosis factor receptor superfamily, member 1a; targeted mutation 1, Tak Mak
Synonyms: Tnfrsf1a-, Pfeffer Tnfrsf1a-, TNFRp55-, TNFR1-, TNFR1/p55-, Tnfrsf1atm1Mak, p55-, TNF-alphaRI-, TNF-R1-, TNFR-
Type: Allele
Species: Mus musculus (mouse)
Chromosome: 6
Alteration at locus: Knockout
Tnfrsf1a
Name: tumor necrosis factor receptor superfamily, member 1a
Synonyms: TNFRI, p55, p55-R, TNF-R55, CD120a, TNFRp55, TNF-alpha-R1, TNFAR, TNFalpha-R1, Tnfr1, TNF-alphaR1, TNF receptor alpha chain, TNF-R1, TNFR60, TNF-R-I
Type: Gene
Species: Mouse
Chromosome: 6
Alteration at locus: Knockout
NCBI: 21937
Homologene: 828
Genetic Alterations

This strain harbors an A>G substitution homologous to a reported case ("IP-TMC") of human incontinentia pigmenti (NM_001099857.3:c.1259A>G/p.(*420Trpext*27) as reported in PMID:11590134). This mouse model reproduces the associated stop codon alteration, but it does not introduce an extension of 27 amino acids as in humans. Instead, it adds, 5' in-frame, an immediate HA tag.

HGVS nomenclature, substitution in the mouse model:

Genotype Determination
ES Cell Line
AB2.2 derived from 129S7/SvEvBrd-Hprtb-m2
Phenotype

Homozygous: embryonic lethality (e12.5)

Heterozygous: skin lesion, eye lesions, and developmental delays in heterozygous females

Mammalian Phenotype Terms
Allelic Composition: Tnfrsf1atm1Mak/Tnfrsf1atm1Mak (Genetic Background: involves: 129S2/SvPas )

Allelic Composition: Tnfrsf1atm1Mak/Tnfrsf1atm1Mak (Genetic Background: involves: 129S2/SvPas * C57BL/6J * DBA/2J )

Allelic Composition: Tnfrsf1atm1Mak/Tnfrsf1atm1Mak (Genetic Background: involves: 129S2/SvPas * C57BL/6J * NOD )

Allelic Composition: Tnfrsf1atm1Mak/Tnfrsf1a+ (Genetic Background: involves: 129S2/SvPas )

MeSH Terms
  • Animals
  • Base Sequence
  • Cell Nucleus/metabolism
  • Cloning, Molecular
  • Humans
  • Immunity, Innate/genetics
  • Kinetics
  • Lipopolysaccharides/toxicity
  • Listeriosis/immunology
  • Listeriosis/physiopathology
  • Liver/pathology
  • Lymphocytes/metabolism
  • Mice
  • Mice, Mutant Strains
  • Molecular Sequence Data
  • Molecular Weight
  • NF-kappa B/metabolism
  • Oligodeoxyribonucleotides
  • Polymerase Chain Reaction/methods
  • Radioligand Assay
  • Receptors, Cell Surface/chemistry
  • Receptors, Cell Surface/genetics
  • Receptors, Cell Surface/metabolism
  • Receptors, Tumor Necrosis Factor
  • Shock, Septic/immunology
  • Shock, Septic/pathology
  • Transfection
  • Tumor Necrosis Factor-alpha/metabolism
  • Tumor Necrosis Factor-alpha/pharmacology
  • Blotting, Northern
  • Blotting, Southern
  • Carrier Proteins
  • Chromosome Aberrations
  • Cohort Studies
  • DNA Primers/chemistry
  • Exons
  • Female
  • Gene Deletion
  • I-kappa B Kinase
  • Incontinentia Pigmenti/enzymology
  • Incontinentia Pigmenti/genetics
  • Male
  • Mitogen-Activated Protein Kinases/genetics
  • Mitogen-Activated Protein Kinases/metabolism
  • Mutation
  • Phenotype
  • Polymerase Chain Reaction
  • Polymorphism, Genetic
  • Reverse Transcriptase Polymerase Chain Reaction
  • X Chromosome/physiology
Strain Development
Ikbkgtm2Dln knock-in mice were backcrossed to C57BL/6J mice to N10+. The mice were then mated with B6.129-Tnfrsf1atm1Mak/J (JAX Strain #002818) for approx. 5 generations, to improve fertility.
Suggested Control Mice
Wild-type littermates
MMRRC Genetic QC Summary
The MMRRC Centers have developed a genetic QC pipeline using MiniMUGA array genotyping to provide additional information on strain backgrounds for MMRRC congenic and inbred strains. For more information on when data may be available, or to request genotyping for a strain of interest, please contact mmrrc@ucdavis.edu. Older strains may not have this information.
  • Apoptosis
  • Cell Biology
  • Dermatology
  • Developmental Biology
  • Hematology
  • Immunology and Inflammation
  • Models for Human Disease
  • Neurobiology
  • Research Tools
Donor
David Nelson, Ph.D., Baylor College of Medicine.
Tak Mak, Ph.D., Campbell Family Institute for Breast Cancer Research.
Primary Reference

Pfeffer K, Matsuyama T, Kündig TM, Wakeham A, Kishihara K, Shahinian A, Wiegmann K, Ohashi PS, Krönke M, Mak TW. Mice deficient for the 55 kd tumor necrosis factor receptor are resistant to endotoxic shock, yet succumb to L. monocytogenes infection. Cell. 1993 May 7;73(3):457-67. (Medline PMID: 8387893)

Aradhya S, Woffendin H, Jakins T, Bardaro T, Esposito T, Smahi A, Shaw C, LevyM, Munnich A, D'Urso M, Lewis RA, Kenwrick S, Nelson DL. A recurrent deletion in the ubiquitously expressed NEMO (IKK-gamma) gene accounts for the vast majorityof incontinentia pigmenti mutations. Hum Mol Genet. 2001 Sep 15;10(19):2171-9.(Medline PMID: 11590134)

Colony and Husbandry Information

The donor maintained these mice on the B6.129-Tnfrsf1atm1Mak/J (JAX Strain #002818) background to improve survival rates and minimize skin lesions. Weaning was done at 4 weeks as the pups are smaller than usual.

Colony Surveillance Program and Current Health Reports

Mice recovered from a cryo-archive will have health surveillance performed on recipient females. Health reports will be provided prior to shipment. If you require additional health status information, please email mmrrc@ucdavis.edu.
Coat Color
Black
Eye
Black
MMRRC Breeding System
Backcross
Breeding Scheme(s)
Wild-type (C57BL/6) female x Hemizygous male or reciprocal mating
Generation
N10+ (C57BL/6J) for Ikbkgtm2Dln allele, then approx. five crosses to B6.129-Tnfrsf1atm1Mak/J (JAX Strain #002818).
Overall Breeding Performance
Good
NOTE: "Hemizygote" as used here refers to males carrying a mutation on the X Chromosome or mice of either sex carrying an inserted transgene with no homologous allele on the other chromosome.
Viability and Fertility: Female Male Comments
Homozygotes are viable: No No
Homozygotes are fertile: N/A N/A
Hetero/Hemizygotes are fertile: Yes N/A
Age Reproductive Decline: >12 months Undetermined
Average litter size
4-6
Recommended wean age
4 weeks
Average Pups Weaned
<4

Order Request Information

Limited quantities of breeder mice (recovered litter) are available from a cryoarchive; recovered litter usually available to ship in 3 to 4 months.

Cryopreserved material may be available upon request, please inquire to mmrrc@ucdavis.edu for more information.

Distribution of this strain requires submission of the MMRRC Conditions of Use (COU). A link to the COU web form will be provided via email after an order has been placed; the form should be completed then or the email forwarded to your institutional official for completion.

The donor or their institution limits the distribution to non-profit institutions only.

Additional charges may apply for any special requests. Shipping costs are in addition to the basic distribution/resuscitation fees. Information on shipping costs and any additional charges will be provided by the supplying MMRRC facility.

Click button to Request this one strain. (Use the MMRRC Catalog Search to request more than one strain.)
MMRRC Item # Description Distribution Fee / Unit (US $)
*Shipping & Handling not included*
Units Notes
041407-UCD-EMBRYO Cryo-preserved embryos $1,038.00 / Non-Profit Aliquot Approximate quantity2 : 20-40 embryos / aliquot
041407-UCD-RESUS Litter recovered from cryo-archive $4,044.00 / Non-Profit Litter Recovered litter4; additional fees for any special requests.
Cryopreserved material may be available upon request, please inquire to mmrrc@ucdavis.edu for more information.

1 The distribution fee covers the expense of rederiving mice from a live mouse; you will receive the resulting litter. The litter will contain at minimum one mutant carrier; the actual number of animals and the gender and genotype ratios will vary. (Typically, multiple breeder pairs can be established from the recovered litter.) Prior to shipment, the MMRRC will provide information about the animals recovered. If you anticipate or find that you need to request specific genotypes, genders or quantities of mice in excess of what is likely from a resuscitated litter, you may discuss available options and pricing with the supplying MMRRC facility.

2 An aliquot contains a sufficient number of embryos (in one or more vials or straws and based on the transfer success rate of the MMRRC facility) to transfer into one to three recipients. The MMRRC makes no guarantee concerning embryo transfer success experienced in the recipient investigator's laboratory. Neither gender nor genotype ratios are guaranteed.

3 An aliquot is one straw or vial with sufficient sperm to recover at least one litter of mice, as per provided protocols, when performed at the MMRRC facility. The MMRRC makes no guarantee concerning the success of these procedures when performed outside the MMRRC facilities.

4 The distribution fee covers the expense of resuscitating mice from the cryo-archive; you will receive the resulting litter. The litter will contain at minimum one mutant carrier; the actual number of animals and the gender and genotype ratios will vary. (Typically, multiple breeder pairs can be established from the recovered litter.) Prior to shipment, the MMRRC will provide information about the animals recovered. If you anticipate or find that you need to request specific genotypes, genders or quantities of mice in excess of what is likely from a resuscitated litter, you may discuss available options and pricing with the supplying MMRRC facility.

To request material from the MMRRC: Please fill out our on-line request form (accessible from the catalog search results page, or click the Request this Strain button in the fees section). If you have questions or need assistance completing this form, you may call Customer Service at (800) 910-2291 (in USA or Canada) or (530) 757-5710 (international calls). Before you call, please have with you: the MMRRC item number, quantity needed, Bill-to and Ship-to contact information.