Overview

This faculty strain has been discontinued but is available as Stock No. 021571.

The T and B cell deficiency in Rag1^tm1Mom null homozygotes prevents manifestation of the spontaneous lupus-like autoimmune syndrome normally observed in BXSB/MpJ males. This strain has been used as a bone marrow transplantation host for investigating the specific lymphocyte or bone marrow populations that transfer autoimmune disease.

Genetic overview

Genetic Background	Generation
000740 BXSB/MpJ

Yaa

Allele Type	Gene Symbol	Gene Name
Spontaneous	Yaa	accelerated autoimmunity and lymphoproliferation transposition

Rag1^tm1Mom

Allele Type	Gene Symbol	Gene Name
Targeted (Null/Knockout)	Rag1	recombination activating gene 1

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Research Applications

Immunology, Inflammation and Autoimmunity Research
Research Tools
Internal/Organ Research
Cancer Research
Hematological Research

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Details

Detailed Description

BXSB/MpJ inbred males (Stock No. 000740) develop a spontaneous lupus-like autoimmune syndrome: mortality starts at approximately 13 weeks of age with 50% lethality by approximately 30 weeks and 76% lethality by approximately 40 weeks. BXSB/MpJ inbred females develop a greatly attenuated form of autoimmune disease because they lack Yaa. Rag1^tm1Mom heterozygotes on this BXSB/MpJ congenic background develop the autoimmune syndrome, but homozygous males, which fail to produce mature T and B cells, are protected from the autoimmunity. This strain has been used for transplant studies to delineate the cell types responsible for the disease onset and regulation.

Development

The Rag1^tm1Mom null allele was backcrossed from B6.129S7-Rag1^tm1Mom/J (Stock No. 002216) onto BXSB/MpJ in the laboratory of Dr. Derry Roopenian. After 11 generations of backcrossing this strain was sibling intercrossed to homozygosity. Male mice carry the Yaa translocation on Chromosome Y. A subline of this strain was frozen as Stock No. 021571.

Selected References

Mombaerts P; Iacomini J; Johnson RS; Herrup K; Tonegawa S; Papaioannou VE. 1992. RAG-1-deficient mice have no mature B and T lymphocytes. Cell 68(5):869-77PubMed: 1547488 MGI: J:1934

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Genetics

Yaa

Allele Symbol: Yaa

Allele Name	accelerated autoimmunity and lymphoproliferation
Allele Type	Spontaneous
Allele Synonym(s)	Is(XOfd1-Mid1;Y)1Mp; Tp(X;Y)1Ekw
Gene Symbol and Name	Yaa, accelerated autoimmunity and lymphoproliferation transposition
Gene Synonym(s)
Strain of Origin	SB/Le
Chromosome	Y
Molecular Note	An approximately 4 MB region of the X chromosome that includes at least 13 known genes (spanning from Ofd1 to Mid1) was translocated to the Y chromosome adjacent to the pseudoautosomal region. Increased RNA expression of Msl3, Tlr7, Tmsb4x and Rab9 was detected in follicular B cells.

Rag1^tm1Mom

Allele Symbol: Rag1^tm1Mom

Allele Name	targeted mutation 1, Peter Mombaerts
Allele Type	Targeted (Null/Knockout)
Allele Synonym(s)	Rag-; Rag^1tm1Mom; RAG-1-; Rag1^-; RAG1^null; Rag-1KO
Gene Symbol and Name	Rag1, recombination activating gene 1
Gene Synonym(s)
Site of Expression	expression is seen in bone marrow derived cell lines.
Strain of Origin	129S7/SvEvBrd-Hprt⁺
Chromosome	2
Molecular Note	A 1356 bp genomic fragment of the Rag1 gene, encoding the nuclear localization signal and the zinc-finger motif, was replaced by a neomycin cassette. A mutant transcript expressed from this allele was detected by Northern blot in bone marrow derived cell lines from homozygous mice.
Mutations Made By	Peter Mombaerts, Max Planck Research Unit for Neurogenetics

Disease/Phenotype

Disease Terms

Research Areas By Phenotype

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

Genotype: Yaa related

Immunology, Inflammation and Autoimmunity Research
- Autoimmunity
  - lupus erythematosus

Genotype: Rag1^tm1Mom related

Research Tools
- Cancer Research
  - B and T cell deficiency, xenograft/transplant host
- Toxicology Research
  - xenograft/transplant host
Internal/Organ Research
- Lymphoid Tissue Defects
  - B and T cell deficiency
Cancer Research
- Toxicology
  - B and T cell deficiency, xenograft/transplant host
Hematological Research
- Immunological Defects
  - B and T cell deficiency
Immunology, Inflammation and Autoimmunity Research
- Immunodeficiency
  - B and T cell deficiency
- T Cell Receptor Signaling Defects
  - B and T cell deficiency
- Inflammation
  - B and T cell deficiency

Immunology, Inflammation and Autoimmunity Research
- Lymphoid Tissue Defects
- Autoimmunity
  - B and T cell deficiency
  - lupus erythematosus, control
Research Tools
- Immunology, Inflammation and Autoimmunity Research

Mammalian Phenotype Terms by Genotype

References

Mombaerts P; Iacomini J; Johnson RS; Herrup K; Tonegawa S; Papaioannou VE. 1992. RAG-1-deficient mice have no mature B and T lymphocytes. Cell 68(5):869-77PubMed: 1547488 MGI: J:1934

Additional - Rag1^tm1Mom related

Additional - Yaa related

Technical Support

CONTACT TECHNICAL SUPPORT

Genotyping Protocols
- Genotyping resources and troubleshooting
Breeding Considerations

To maintain the live colony, homozygous mice may be bred together. Homozygous animals of both sexes do not develop the spontaneous lupus-like autoimmune syndrome observed for BXSB/MpJ inbred mice (Stock No. 000740). Homozygous mice are lymphocyte-deficient. As such, they should be maintained in pathogen-free conditions on sulfatrim water to increase colony health (similar to other immunodeficient strains). Heterozygotes will develop the sex-specific autoimmune phenotype of BXSB/MpJ. The expected coat color is white-bellied agouti.
- Additional Breeding and Husbandry Support
Mating System
- Homozygote x Homozygote

Terms Of Use

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Questions about Terms of Use

Additional Use Restrictions Apply

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Licensing Information

Phone: 207-288-6470

Email: TechTran@jax.org

Genetic overview

Research Applications

Detailed Description

Development

Selected References

Yaa

Allele Symbol: Yaa

Rag1tm1Mom

Allele Symbol: Rag1tm1Mom

Disease Terms

Research Areas By Phenotype

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

Genotype: Yaa related

Genotype: Rag1tm1Mom related

Mammalian Phenotype Terms by Genotype

References

Additional - Rag1tm1Mom related

Additional - Yaa related

Genotyping Protocols

Breeding Considerations

Mating System

Terms Of Use

Additional Use Restrictions Apply

Licensing Information

Rag1^tm1Mom

Allele Symbol: Rag1^tm1Mom

Genotype: Rag1^tm1Mom related

Additional - Rag1^tm1Mom related