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General Terms and Conditions

Former Name	CBA/KlJms-Tnfrsf6lpr-cg/J    (Changed: 26-JAN-05 )
	CBA    (Changed: 15-DEC-04 )
Genes & Alleles	Fas;   Faslpr-cg;

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Product Information

Strain Details

Type JAX® GEMM® Strain - Mutant Strain Additional information on JAX® GEMM® Strains. Type JAX® GEMM® Strain - Spontaneous Mutation Mating System Homozygote x Homozygote         (Female x Male) Species laboratory mouse Generation F49 (24-JUL-07)

Appearance
agouti
Related Genotype: A/A

Strain Description
Mice homozygous for the lymphoproliferation complementing gld spontaneous mutation (Fas^lpr-cg) are viable and fertile. Homozygous mutant mice are characterized by massive lymphadenopathy. Fas^lpr-cg complemented both Fas^lpr and the Fasl^gld mutation in that double heterozygotes with either mutation had lymphadenopathy. However, further crosses showed the new mutation to be an allele at Fas^lpr. Like Fas^lpr and Fasl^gld homozygotes, CBA/KlJms-Fas^lpr-cg homozygotes produce antibodies to some nuclear antigens, such as dsDNA, ssDNA, and poly(ADP-ribose); however, they do not produce anti-erythrocyte antibodies. Although they exhibit lymphoid cell infiltration around blood vessels in lung, liver, and kidney, they lack the immune-complex glomerulonephritis, vasculitis, and interstitial pneumonia characteristic of Fas^lpr homozygotes.

Strain Development
The Fas^lpr-cg mutation occurred spontaneously in a subline of the inbred strain CBA/KlJms maintained at the Institute of Medical Science in Tokyo. Fas^lpr-cg complemented both the Fas^lpr and the Fasl^gld mutations in that double heterozygotes with either mutation had lymphadenopathy.

Related Disease (OMIM) Terms Autoimmune Lymphoproliferative Syndrome; ALPS

Mammalian Phenotype Terms assigned by genotype Faslpr-cg/Faslpr-cg         CBA/KlJms-Faslpr-cg/J life span-post-weaning/aging premature death (MGI Ref ID J:24805) earliest deaths at 19 and 29 weeks of age survival rate at 1 year is 61.1% in males and 46.2% in females immune system phenotype abnormal T cell differentiation (MGI Ref ID J:24805) anomalous differentiation of T cells, with anomalous expression of surface antigens abnormal lymph organ size (MGI Ref ID J:24805) enlarged lymph nodes (MGI Ref ID J:81770) enlargement of the superficial lymph nodes starts at about 2.5 months of age, with cervical preceding inguinal lymph nodes superficial lymph nodes are more than 5 and 15 times heavier than controls at 3 and 5 months of age, respectively mesenteric lymph nodes are normal in size enlarged spleen (MGI Ref ID J:24805) palpable by 3 months of age increased spleen weight (MGI Ref ID J:81770) spleens larger in females than males spleen weights increase up to 6 months of age and remain at a plateau thereafter autoimmune response (MGI Ref ID J:24805) increased autoantibody level (MGI Ref ID J:81770) increased anti-nuclear antigen antibody level (MGI Ref ID J:24805) elevated levels of autoantibodies to nuclear antigens including anti-DNA hypersensitivity (MGI Ref ID J:81770) lymphocyte infiltration in liver, lungs, spleen, and kidneys increased immunoglobulin level (MGI Ref ID J:24805) increased IgG level (MGI Ref ID J:24805) five times higher than in controls increased IgM level (MGI Ref ID J:24805) two times higher than in controls hematopoietic system phenotype abnormal T cell differentiation (MGI Ref ID J:24805) anomalous differentiation of T cells, with anomalous expression of surface antigens enlarged spleen (MGI Ref ID J:24805) palpable by 3 months of age increased spleen weight (MGI Ref ID J:81770) spleens larger in females than males spleen weights increase up to 6 months of age and remain at a plateau thereafter

Gene & Allele Details

Allele Symbol		Faslpr-cg
Allele Name		lymphoproliferation complementing gld
Common Name(s)		lprcg;
Strain of Origin		CBA/KlJms
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		This mutation, which produces massive lymphadenopathy in homozygotes, occurred spontaneously in a subline of the inbred strain CBA/KlJms maintained at the Institute of Medical Science in Tokyo. Faslpr-cg complemented both Faslpr andthe generalized lymphoproliferative disease Faslgld mutation in that double heterozygotes with either mutation had lymphadenopathy. However, further crosses showed the new mutation to be an allele at Faslpr (J:24805). Like Faslpr and Faslgld mutant homozygotes, CBA-Faslpr-cg/Faslpr-cg mutants produce antibodies to some nuclear antigens, such as dsDNA, ssDNA, and poly(ADP-ribose); however, they do not produce anti-erythrocyte antibodies. Although they exhibit lymphoid cell infiltration around blood vessels in lung, liver, and kidney, they lack the immune-complex glomerulonephritis, vasculitis, and interstitial pneumonia characteristic of Faslpr homozygotes (J:616). Faslpr-cg homozygotes on the MRL genetic background developed glomerulonephritic lesions similar to those of MRL/MpJ-Faslpr mutants, although at a lower frequency, suggesting MRL/MpJ background genes control this aspect of the disease (J:1753). Studies of Faslpr and Faslpr-cg homozygotes in athymic nude (Hfh11nu/Hfh11nu) mice show that the thymus is critical for lymphadenopathy and autoimmunity (J:582).
Molecular Note		A T-to-A transversion point mutation at nucleotide 786 results in replacement by asparagine of a highly conserved isoleucine in the cytoplasmic region of the encoded protein. [MGI Ref ID J:1181]

Control Information

Allele	Control
Faslpr-cg	000654 CBA/CaJ	(approximate)
		The recommended control for this strain has been changed from CBA/J to CBA/CaJ because 3 out of 28 Single Nucleotide Polymorphism (SNP) markers and 3 out of 25 isoenzyme markers were shown to differ between CBA/KlJms-Faslpr-cg/J and CBA/J. However CBA/KlJms-Faslpr-cg/J and CBA/CaJ type the same for the markers in question.
Considerations for Choosing Controls

Colony Maintenance

Diet Information	LabDiet® 5K52/5K67

Related Strains

Strains carrying   Fas^lpr-cg allele

002983

MRL.CBAJms-Faslpr-cg/J

View Strains carrying   Fas^lpr-cg     (1 strain)

Strains carrying other alleles of Fas

003233	B6.129P2-Fastm1Osa/J
000482	B6.MRL-Faslpr/J
000480	C3.MRL-Faslpr/J
007895	C57BL/6-Fastm1Cgn/J
002455	MRL-Faslpr.129P2(B6)-B2mtm1Unc
003234	MRL.129P2(B6)-Fastm1Osa/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)

Animal Health Reports

Room Number           AX11

Research Applications

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

Fas^lpr-cg related

Apoptosis Research
Death Receptors

Cancer Research
Genes Regulating Growth and Proliferation

Immunology and Inflammation Research
Autoimmunity (lupus erythematosus)
Inflammation

Mouse/Human Gene Homologs
autoimmune lymphoproliferative syndrome

References

Selected Reference(s)

Kimura M; Ogata Y; Shimada K; Moriyama T; Matsuzawa A. 1991. New mutant mice of autoimmunity, CBA/KiJms-lprcg/lprcg, that could link the lpr and gld genes [letter] Autoimmunity 9(4):359-61. [PubMed: 1954317]  [MGI Ref ID J:616]

Kimura M; Ogata Y; Shimada K; Wakabayashi T; Onoda H; Katagiri T; Matsuzawa A. 1992. Nephritogenicity of the lprcg gene on the MRL background. Immunology 76(3):498-504. [PubMed: 1526655]  [MGI Ref ID J:1753]

Matsuzawa A; Moriyama T; Kaneko T; Tanaka M; Kimura M; Ikeda H; Katagiri T. 1990. A new allele of the lpr locus, lprcg, that complements the gld gene in induction of lymphadenopathy in the mouse. J Exp Med 171(2):519-31. [PubMed: 2406366]  [MGI Ref ID J:24805]

Additional References

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Price and Supply Information

Strain Name:	CBA/KlJms-Faslpr-cg/J
Stock Number:	001876

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Supply Details

Standard Supply	Repository-Live. A collection of over 1000 strains maintained as live colonies. Individual colonies are sized to meet current customer demand. Delivery for orders of 10 mice or less ranges on average from one to eight weeks; mice are generally shipped between four to six weeks of age with a maximum shipping age of ~nine weeks. Colony sizes do not generally support stringent age specifications for large volumes of mice; however custom orders and larger quantities of mice are easily arranged. Estimated ship dates for all orders provided within 48 hours of order placement.
Supply Notes	Usually shipped between four and eight weeks of age. This strain is included in the Mouse Mutant Resource collection.
Licensing	See General Terms and Conditions below
Control Information	View Control Information in Strain Details.

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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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