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Strain Name:

MRL.129P2(B6)-Fastm1Osa/J

Stock Number:

003234

Availability:

Repository-Cryopreserved

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General Terms and Conditions

Former Name      MRL.129P2(B6)-Tnfrsf6tm1Osa/J    (Changed: 26-JAN-05 )
Genes & Alleles   Fas;   Fastm1Osa;

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
Donor Strain B6,129P-Tnfrsf6tm1Osa (129P2 derived E14TG2a ES cell line)
Donating Investigator Shigekazu Nagata,   Osaka University Medical School, B-3
GenerationF?+4N1F1pN1

Strain Description
Mice homozygous for the Tnfrsf6tm1Osa targeted mutation mice show hyperplasia in the liver in an age-dependent manner. Hepatocytes contain enlarged nuclei characteristic of senescent cells. Lymphadenopathy and splenomegaly in FAS deficient mice are evident at 8 weeks of age. The lymphnodes and spleen become progressively larger, becoming about 10 and 40 times larger than those of wildtype or heterozygotes at 16 weeks of age, respectively.

Strain Development
The targeting vector was constructed by replacing exon 9 of the Fas gene with the neo and beta-galactosidase genes. E14-1 ES cells from 129/Ola mice were disrupted by homologous recombination and injected into blastocysts isolated from C57BL/6 mice.

Related Disease (OMIM) Terms

Autoimmune Lymphoproliferative Syndrome; ALPS
Mammalian Phenotype Terms assigned by genotype

The following phenotype information may relate to a genetic background differing from this JAX® Mice strain.

Fastm1Osa/Fastm1Osa

        involves: 129P2/OlaHsd * C57BL/6
  • life span-post-weaning/aging
  • premature death (MGI Ref ID J:64296)
    • 50% dead at about 5 months of age
  • liver/biliary system phenotype
  • abnormal hepatocyte morphology (MGI Ref ID J:64296)
    • hepatocyte nuclear areas are variable and larger
  • liver hyperplasia (MGI Ref ID J:64296)
    • significantly heavier at 12 weeks of age and becomes 50-60% heavier by 16 weeks of age
    • number of hepatocytes per each liver lobe is increased
  • immune system phenotype
  • abnormal T cell clonal deletion (MGI Ref ID J:32125)
    • peripheral clonal deletion of mature T cells against a bacterial superantigen is impaired
  • abnormal lymph organ size (MGI Ref ID J:64296)
    • enlarged lymph nodes (MGI Ref ID J:32125)
      • lymph nodes become progressively larger
    • enlarged spleen (MGI Ref ID J:32125)
      • spleen becomes progressively larger
    • lymphoid hyperplasia (MGI Ref ID J:64296)
  • hypersensitivity (MGI Ref ID J:32125)
    • occasional infiltration of lymphocytes into tissues such as the lungs and liver
  • increased IgG level (MGI Ref ID J:32125)
    • 10-100x higher serum levels of IgG1, IgG2a, IgG2b, and IgG3 at 16 weeks of age
    • increased IgG1 level (MGI Ref ID J:32125)
    • increased IgG2a level (MGI Ref ID J:32125)
    • increased IgG2b level (MGI Ref ID J:32125)
    • increased IgG3 level (MGI Ref ID J:32125)
  • increased autoantibody level (MGI Ref ID J:32125)
    • 50-500x higher levels of dsDNA and ssDNA autoantibodies at 16 weeks of age
    • increased anti-double stranded DNA antibody level (MGI Ref ID J:32125)
    • increased anti-single stranded DNA antibody level (MGI Ref ID J:32125)
  • increased leukocyte cell number (MGI Ref ID J:32125)
    • 20x higher than in controls, attributable to increased lymphocyte numbers
    • increased lymphocyte cell number (MGI Ref ID J:32125)
      • lymphocytosis; progressively accumulate abnromal T cells Thy1+ B220+ CD4- CD8 -
      • increased B cell number (MGI Ref ID J:32125)
        • increase in the number of normal B cells
  • increased susceptibility to parasitic infection (MGI Ref ID J:81397)
    • decreased survival after infection with Trypanosoma cruzi even though were able to clear parasites from blood and affected organs
  • hematopoietic system phenotype
  • abnormal T cell clonal deletion (MGI Ref ID J:32125)
    • peripheral clonal deletion of mature T cells against a bacterial superantigen is impaired
  • enlarged spleen (MGI Ref ID J:32125)
    • spleen becomes progressively larger
  • increased leukocyte cell number (MGI Ref ID J:32125)
    • 20x higher than in controls, attributable to increased lymphocyte numbers
    • increased lymphocyte cell number (MGI Ref ID J:32125)
      • lymphocytosis; progressively accumulate abnromal T cells Thy1+ B220+ CD4- CD8 -
      • increased B cell number (MGI Ref ID J:32125)
        • increase in the number of normal B cells

Gene & Allele Details

Allele Symbol Fastm1Osa
Allele Name targeted mutation 1, Osaka University Medical School
Common Name(s) Fas-; Tnfrsf6tm1Osa;
Mutation Made By Shigekazu Nagata,   Osaka University Medical School, B-3
Strain of Origin129P2/OlaHsd
ES Cell Line NameE14.1
ES Cell Line Strain129P2/OlaHsd
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;
Molecular Note A BetaGal-neo cassette was used to delete a portion of exon 9, which codes for the cytoplasmic domain essential for Fas-mediated apoptosis. Northern blot analysis revealed the expression of a truncated mRNA, representing transcripts from exons 1 though 8 in the thymus, liver, and heart. Immunoprecipitation analysis showed an absence of the wild-type protein as well as a faint presence of what the authors speculated to be a non-functional Tnfrsf6-BetaGal fusion protein. [MGI Ref ID J:64296]

Control Information

   
   MRL/MpJ (Stock # 000485) may be used as controls.
 
  Considerations for Choosing Controls

Genotyping Protocols

Fastm1Osa

Related Strains

Strains carrying   Fastm1Osa allele
003233   B6.129P2-Fastm1Osa/J
View Strains carrying   Fastm1Osa     (1 strain)

Strains carrying other alleles of Fas
000482   B6.MRL-Faslpr/J
000480   C3.MRL-Faslpr/J
007895   C57BL/6-Fastm1Cgn/J
001876   CBA/KlJms-Faslpr-cg/J
002455   MRL-Faslpr.129P2(B6)-B2mtm1Unc
002983   MRL.CBAJms-Faslpr-cg/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 other alleles of Fas     (11 strains)

Additional Web Information

Congenic Nomenclature

Research Applications

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

Fastm1Osa related

Apoptosis Research
Death Receptors

Cancer Research
Genes Regulating Growth and Proliferation

Hematological Research
Immunological Defects

Immunology and Inflammation Research
Autoimmunity
CD Antigens, Antigen Receptors, and Histocompatibility Markers

Internal/Organ Research
Spleen Defects

References

Selected Reference(s)

Adachi M; Suematsu S; Kondo T; Ogasawara J; Tanaka T; Yoshida N; Nagata S. 1995. Targeted mutation in the Fas gene causes hyperplasia in peripheral lymphoid organs and liver. Nat Genet 11(3):294-300. [PubMed: 7581453]  [MGI Ref ID J:64296]

Additional References

Price and Supply Information

Strain Name: MRL.129P2(B6)-Fastm1Osa/J
Stock Number: 003234

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.
This strain is included in the Induced Mutant Resource Colony collection.
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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