This faculty strain has been discontinued but is available as Stock No. 021571.
The T and B cell deficiency in Rag1tm1Mom 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.
Read More +Genetic Background | Generation |
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000740 BXSB/MpJ |
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Allele Type | Gene Symbol | Gene Name |
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Spontaneous | Yaa | accelerated autoimmunity and lymphoproliferation transposition |
Allele Type | Gene Symbol | Gene Name |
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Targeted (Null/Knockout) | Rag1 | recombination activating gene 1 |
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. Rag1tm1Mom 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.
The Rag1tm1Mom null allele was backcrossed from B6.129S7-Rag1tm1Mom/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.
Allele Name | accelerated autoimmunity and lymphoproliferation |
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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. |
Allele Name | targeted mutation 1, Peter Mombaerts |
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Allele Type | Targeted (Null/Knockout) |
Allele Synonym(s) | Rag-; Rag1tm1Mom; RAG-1-; Rag1-; RAG1null; 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 |
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.
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