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Strain Name:

MRL-Faslpr.129P2(B6)-B2mtm1Unc

Stock Number:

002455

Availability:

Repository-Cryopreserved

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General Terms and Conditions

Former Name      MRL-Tnfrsf6lpr.129P2(B6)-B2mtm1Unc    (Changed: 26-JAN-05 )
Genes & Alleles   B2m;   B2mtm1Unc;   Fas;   Faslpr;

Product Information

Strain Details

Type JAX® GEMM® Strain - Congenic
Additional information on JAX® GEMM® Strains.
Type JAX® GEMM® Strain - Mutant Strain
Type JAX® GEMM® Strain - Targeted Mutation
Specieslaboratory mouse
Background Strain MRL/MpJ-Faslpr/J
Donor Strain B6;129P-B2mtm1Unc (129P2 derived E14TG2a ES cell line)
Donating Investigator Derry Roopenian,   The Jackson Laboratory
GenerationN11F10p

Appearance
B2m: albino
Related Genotype: a/a Tyrc/Tyrc

Faslpr: albino, affected
Related Genotype: a/a B2mtm1Unc/B2mtm1Unc Tyrc/Tyrc Faslpr/Faslpr

Strain Description
Mice homozygous for both the lymphoproliferation spontaneous mutation (Faslpr) and the B2mtm1Unc targeted mutation are viable and fertile. Class I molecule-deficient B2mtm1Unc mice were backcrossed to MRL/MpJ-Faslpr to study the role of MHC class I molecules in the development of systemic autoimmune disease. Double homozygous mutant mice demonstrated a substantial reduction in CD4-CD8- T cells and a diminution of autoimmune disease. Specifically, hypergammaglobulinemia; autoantibodies including anti-DNA, anti-Smith antigen, and rheumatoid factor; and glomerulonephritis were significantly reduced compared to MRL/MpJ-Faslpr homozygous mice. Moreover, the pattern of hypergammaglobulinemia suggests that the requirement for MHC class I proteins is restricted temporally to later stages of the disease.

Strain Development
The B2mtm1Unc mutant strain was developed in the laboratory of Dr. Beverly Koller and Dr. Oliver Smithies at the University of North Carolina at Chapel Hill. It was generated by a targeted disruption of the B2m gene. The 129-derived E14TG2a ES cell line was used. The MRL-Faslpr .129P2(B6)-B2mtm1Unc strain was produced in the laboratory of Dr. Derry Roopenian at The Jackson Laboratory by backcrossing the B2mtm1Unc mutation 10 times to MRL/MpJ-Faslpr inbred mice.

Gene & Allele Details

Allele Symbol B2mtm1Unc
Allele Name targeted mutation 1, University of North Carolina
Common Name(s) I0; b2mnull; beta2-m-KO; beta2M-; beta2MKO; beta2m0; beta2mnull; beta2mo; beta2mtm1Unc;
Mutation Made By Oliver Smithies,   University of North Carolina
Strain of Origin129P2/OlaHsd
ES Cell Line NameE14TG2a
ES Cell Line Strain129P2/OlaHsd
Gene Symbol and Name B2m, beta-2 microglobulin
Chromosome 2
Gene Common Name(s) Ly-m11; beta 2 microglobulin; beta2-m; lymphocyte antigen m11;
Molecular Note Insertion of a neomycin-resistance gene into the second exon. [MGI Ref ID J:65612]
 
Allele Symbol Faslpr
Allele Name lymphoproliferation
Common Name(s) Fas-; Tnfrf6lpr; Tnfrsf6lpr; Tnfrsf6lpr; lpr;
Strain of OriginMRL/Mp
Gene Symbol and Name Fas, Fas (TNF receptor superfamily member 6)
Chromosome 19
Gene Common Name(s) AI196731; ALPS1A; APO-1; APT1; CD95; FAS1; FASTM; Fas antigen; TNFR6; TNFRSF6; Tnfrsf6; expressed sequence AI196731; lpr; lymphoproliferation; tumor necrosis factor receptor superfamily, member 6;
General Note Faslpr, lymphoproliferation, recessive. This mutation was found during inbreeding of a strain MRL/Mp derived from crosses among strains LG, AKR, C3H, and C57BL/6. The resemblance has led to extensive use of Faslpr mice in attemptsto determine the etiology of SLE and to evaluate therapies. However, the human APT1 gene (OMIM 134637) encodes the FAS antigen; Tnfrsf6 is not the homolog of the human (SLE) gene.
Molecular Note Southern blotting experiments indicated that the mutation is a genomic rearrangement within the gene, probably within intron 2. [MGI Ref ID J:1181] [MGI Ref ID J:14206] [MGI Ref ID J:14503] [MGI Ref ID J:15429] [MGI Ref ID J:4166] [MGI Ref ID J:4342] [MGI Ref ID J:72675] [MGI Ref ID J:92470]

Control Information

  Allele   Control
 B2mtm1Unc  000485 MRL/MpJ-Faslpr/J
 Faslpr  000485 MRL/MpJ-Faslpr/J
 
  Considerations for Choosing Controls

Genotyping Protocols

B2mtm1Unc
Faslpr

Colony Maintenance

Breeding & HusbandryThis double mutant strain is maintained by mating homozygous siblings. Only homozygous mice may be ordered. Expected coat color from breeding: albino

Related Strains

View Strains carrying   B2mtm1Unc     (19 strains)

Strains carrying   Faslpr allele
000482   B6.MRL-Faslpr/J
000480   C3.MRL-Faslpr/J
003896   MRL/MpJ Faslpr-Foxq1sa-J/J
006825   MRL/MpJ-Faslpr/2J
000485   MRL/MpJ-Faslpr/J
004519   NOD.MRL(C3)-Faslpr/DoiJ
004922   NOD.MRL-Faslpr/Dvs
View Strains carrying   Faslpr     (7 strains)

Strains carrying other alleles of Fas
003233   B6.129P2-Fastm1Osa/J
007895   C57BL/6-Fastm1Cgn/J
001876   CBA/KlJms-Faslpr-cg/J
003234   MRL.129P2(B6)-Fastm1Osa/J
002983   MRL.CBAJms-Faslpr-cg/J
View Strains carrying other alleles of Fas     (5 strains)

Additional Web Information

Congenic Nomenclature

Research Applications

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

B2mtm1Unc related

Hematological Research
Anemia, Iron Deficiency and Transport Defects (hemochromatosis)

Immunology and Inflammation Research
Immunodeficiency (MHC class I deficiency)

Internal/Organ Research
Liver Defects (hemochromatosis)

Metabolism Research
Hemochromatosis (iron metabolism defects)

Research Tools
Immunology and Inflammation Research (MHC class I deficiency)

Faslpr related

Apoptosis Research
Death Receptors

Cancer Research
Genes Regulating Growth and Proliferation

Immunology and Inflammation Research
Autoimmunity (lupus erythematosus: rheumatoid arthritis)
Inflammation (rheumatoid arthritis)

Mouse/Human Gene Homologs
autoimmune lymphoproliferative syndrome

References

Selected Reference(s)

Koller BH; Marrack P; Kappler JW; Smithies O. 1990. Normal development of mice deficient in beta 2M, MHC class I proteins, and CD8+ T cells. Science 248(4960):1227-30. [PubMed: 2112266]  [MGI Ref ID J:64451]

Additional References

Price and Supply Information

Strain Name: MRL-Faslpr.129P2(B6)-B2mtm1Unc
Stock Number: 002455

Price Details

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Supply Details

Standard SupplyRepository-Cryopreserved. Must Be Recovered. Please refer to pricing and supply notes for further information.
Supply Notes Cryorecovery - Standard.
The recovery process begins when a signed agreement form is returned to the Customer Service Department after order placement. Although results vary by strain, at least two males and two females (two pairs) will be provided, typically within 15 weeks of our receipt of the signed agreement form. If the first recovery attempt is unsuccessful or only one pair is recovered, a second recovery will be done, extending the delivery time to approximately 25 weeks. At least one member of each pair will be of known genotype and will carry the mutation if it is a mutant strain. Please note that pairs may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation of the strain. Mating schemes are sometimes modified for successful cryopreservation. Price represents a repository maintenance fee, which includes the cost of recovery of the strain from the cryopreservation resource and the periodic replacement of the frozen embryos used for recovery.

Cryorecovery to establish a Dedicated Supply for greater quantities of mice.
One to two pairs will be recovered to establish a Dedicated Supply of mice. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services: Tel: 1-800-422-6423 or 1-207-288-5845; Email: jaxservices@jax.org.
Genomic DNA is available for this strain from the Mouse DNA Resource.

LicensingSee General Terms and Conditions below  
Control InformationView Control Information in Strain Details.

General Terms and Conditions

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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.
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