BXSB.129S7(B6)-Rag1^tm1Mom/Dcr

Stock number: 009437
Research areas: Cancer Research, Hematological Research, Immunology, Inflammation and Autoimmunity Research, Internal/Organ Research, Research Tools

Description

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.

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.

Allele

Symbol: Yaa
Name: accelerated autoimmunity and lymphoproliferation
MGI accession ID: MGI:1856277
Generation method: Spontaneous
Marker symbol: Yaa
Marker name: accelerated autoimmunity and lymphoproliferation transposition
Chromosome: Y
Strain of origin: SB/Le
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

Symbol: Rag1^tm1Mom
Name: targeted mutation 1, Peter Mombaerts
MGI accession ID: MGI:1857241
Generation method: Targeted
Attributes: Null/Knockout
Marker symbol: Rag1
Marker name: recombination activating gene 1
Chromosome: 2
Strain of origin: 129S7/SvEvBrd-Hprt⁺
Site of expression: expression is seen in bone marrow derived cell lines.
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.