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Strain Name:

CBA/CaHN-Btkxid/J

Stock Number:

001011

Availability:

Level 2

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General Terms and Conditions

Former Name      CBA/NJ    (Changed: 15-DEC-04 )
Genes & Alleles   Btk;   Btkxid;

Product Information

Strain Details

Type Inbred Strain
Additional information on Inbred Strains.
Type JAX® GEMM® Strain - Mutant Strain
Additional information on JAX® GEMM® Strains.
Mating SystemHomozygote x Hemizygote         (Female x Male)
Specieslaboratory mouse
H2 Haplotypek
GenerationF88 (03-JAN-08)

Appearance
agouti
Related Genotype: A/A

Strain Description
CBA/CaHN-Btkxid/J mice have a mutation in the Bruton's tyrosine kinase gene (Btk), and are a model of human X-linked immunodeficiency. They have a B-lymphocyte-specific defect that results in an inability to launch an antibody response to thymus-independent type II antigens, although they do produce normal amounts of antibody in response to some protein antigens. They have low serum IgM and IgG3 and a reduced number of B-cells. Moreover, the B-cells that are present have a reduced surface IgM to IgD ratio, which suggests a disorder in B-cell maturation.

Mammalian Phenotype Terms assigned by genotype

Btkxid/Btkxid

        CBA/CaHN-Btkxid/J
  • immune system phenotype
  • abnormal macrophage physiology (MGI Ref ID J:92639)
    • macrophages (adherent peritoneal exudates cells, PECs) stimulated with LPS show a reduced induction of ROIs than wild type macrophages at all LPS concentrations
  • abnormal neutrophil physiology (MGI Ref ID J:92639)
    • mutant polymorphonuclear leukocytes (PMNs) show poorer induction of reactive oxygen intermediates (ROIs) upon LPS stimulation compared to wild type; NO production is significantly less in mutant PMNs
    • impaired neutrophil phagocytosis (MGI Ref ID J:92639)
      • phagocytic ability of PMNs is somewhat lower, but not significantly, compared to wild type
  • decreased acute inflammation (MGI Ref ID J:92639)
    • peak footpad swelling in response to carrageenan injection is reduced compared to wild type mice
  • decreased neutrophil cell number (MGI Ref ID J:92639)
    • total leukocyte numbers are not significantly altered compared to wild type, but significantly reduced peripheral PMN frequency in blood is observed in mutants
  • decreased susceptibility to experimental autoimmune encephalomyelitis (MGI Ref ID J:92639)
    • mice show slower induction of experimental autoimmune encephalomyelitis (EAE) and milder clinical disease than wild type mice
  • impaired myelopoiesis (MGI Ref ID J:92639)
    • number of monocytic and granulocytic-lineage cells is significantly reduced in mutant bone marrow
    • frequency of monocytic/granulocytic precursors (myeloid CFUs) is significantly reduced compared to wild type bone marrow
  • increased susceptibility to induced colitis (MGI Ref ID J:92639)
    • dextran sulfate sodium-induced colitis results in significant weight loss in wild type mice by day 10 of DSS treatment while mutants do not exhibit any weight loss at that time
  • hematopoietic system phenotype
  • decreased neutrophil cell number (MGI Ref ID J:92639)
    • total leukocyte numbers are not significantly altered compared to wild type, but significantly reduced peripheral PMN frequency in blood is observed in mutants
  • impaired myelopoiesis (MGI Ref ID J:92639)
    • number of monocytic and granulocytic-lineage cells is significantly reduced in mutant bone marrow
    • frequency of monocytic/granulocytic precursors (myeloid CFUs) is significantly reduced compared to wild type bone marrow
  • digestive/alimentary phenotype
  • increased susceptibility to induced colitis (MGI Ref ID J:92639)
    • dextran sulfate sodium-induced colitis results in significant weight loss in wild type mice by day 10 of DSS treatment while mutants do not exhibit any weight loss at that time

Btkxid/Btkxid

        CBA/HN-Btkxid
  • immune system phenotype
  • abnormal lymphopoiesis (MGI Ref ID J:7981)
    • incidence of small lymphocytes is slightly lower, but absolute population is not, compared to controls
    • abnormal pre-B cell morphology (MGI Ref ID J:7981)
      • incidence of pre-B cells is slightly reduced, but absolute population is comparable to controls
    • decreased B cell proliferation (MGI Ref ID J:81429)
      • cultured B cells show little response to B-cell mitogens LPS and poly I:C in comparison to wild type cells
      • proliferative response in B cells from females is drastically lower than in cells from female mutants on CBA/HN * DBA/2N background
  • abnormal spleen cellularity (MGI Ref ID J:81429)
    • number of nucleated spleen cells (~60 x 106/spleen) is significantly lower than in controls (~120 x 106/spleen)
    • females have significantly less than females on the CBA/HN * DBA/2N background which have (~90 x 106/spleen)
  • autoimmune response (MGI Ref ID J:125114)
    • mice do not develop autoantibodies (anti-dsDNA or any IgG antinuclear autoantibodies)
  • decreased B cell number (MGI Ref ID J:81429)
    • spleens have only ~22% immunoglobulin-bearing cells compared to ~50% in controls
    • numbers (22%) are less than female mutants on CBA/HN * DBA/2N background (40%)
    • incidence of B lymphocytes is slightly lower, but absolute population is not, compared to controls
  • hematopoietic system phenotype
  • abnormal bone marrow cell number (MGI Ref ID J:7981)
    • number of nucleated cells is higher in mutants than in controls
  • abnormal lymphopoiesis (MGI Ref ID J:7981)
    • incidence of small lymphocytes is slightly lower, but absolute population is not, compared to controls
    • abnormal pre-B cell morphology (MGI Ref ID J:7981)
      • incidence of pre-B cells is slightly reduced, but absolute population is comparable to controls
    • decreased B cell proliferation (MGI Ref ID J:81429)
      • cultured B cells show little response to B-cell mitogens LPS and poly I:C in comparison to wild type cells
      • proliferative response in B cells from females is drastically lower than in cells from female mutants on CBA/HN * DBA/2N background
  • abnormal spleen cellularity (MGI Ref ID J:81429)
    • number of nucleated spleen cells (~60 x 106/spleen) is significantly lower than in controls (~120 x 106/spleen)
    • females have significantly less than females on the CBA/HN * DBA/2N background which have (~90 x 106/spleen)
  • decreased B cell number (MGI Ref ID J:81429)
    • spleens have only ~22% immunoglobulin-bearing cells compared to ~50% in controls
    • numbers (22%) are less than female mutants on CBA/HN * DBA/2N background (40%)
    • incidence of B lymphocytes is slightly lower, but absolute population is not, compared to controls

Btkxid/Y

        CBA/HN-Btkxid
  • immune system phenotype
  • abnormal spleen cellularity (MGI Ref ID J:81429)
    • number of nucleated spleen cells (~60 x 106/spleen) is significantly lower than in controls (~120 x 106/spleen)
  • decreased B cell number (MGI Ref ID J:81429)
    • spleens have only ~22% immunoglobulin-bearing cells compared to ~50% in controls
  • decreased B cell proliferation (MGI Ref ID J:81429)
    • cultured B cells show little response to B-cell mitogens LPS and poly I:C in comparison to wild type cells
  • hematopoietic system phenotype
  • abnormal spleen cellularity (MGI Ref ID J:81429)
    • number of nucleated spleen cells (~60 x 106/spleen) is significantly lower than in controls (~120 x 106/spleen)
  • decreased B cell number (MGI Ref ID J:81429)
    • spleens have only ~22% immunoglobulin-bearing cells compared to ~50% in controls
  • decreased B cell proliferation (MGI Ref ID J:81429)
    • cultured B cells show little response to B-cell mitogens LPS and poly I:C in comparison to wild type cells

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

Btkxid/Btkxid

        involves: CBA/HN * DBA/2N
  • hematopoietic system phenotype
  • abnormal B cell proliferation (MGI Ref ID J:81429)
    • B cells show response to LPS and poly I:C similar to wild type cells
    • proliferative response in B cells from females is much higher than in cells from female mutants on CBA/HN background
  • abnormal spleen cellularity (MGI Ref ID J:81429)
    • number of nucleated spleen cells (~90 x 106/spleen) is significantly lower than in controls (~120 x 106/spleen)
    • females on the CBA * DBA/2J (~90 x 106/spleen) have significantly more nucleated spleen cells CBA/HN females (~60 x 106/spleen)
  • decreased B cell number (MGI Ref ID J:81429)
    • spleens from females have ~40% immunoglobulin-bearing cells compared to ~50% in controls
    • numbers are greater in female mutants on CBA * C57BL/6 background (40%) than females on CBA/HN background (22%)
  • immune system phenotype
  • abnormal B cell physiology (MGI Ref ID J:81429)
    • spleen cells from unsensitized females cause 3-fold higher chromium release from H-2b antibody treated EL-4 tumor cells than splenocytes from males
    • abnormal B cell proliferation (MGI Ref ID J:81429)
      • B cells show response to LPS and poly I:C similar to wild type cells
      • proliferative response in B cells from females is much higher than in cells from female mutants on CBA/HN background
  • abnormal spleen cellularity (MGI Ref ID J:81429)
    • number of nucleated spleen cells (~90 x 106/spleen) is significantly lower than in controls (~120 x 106/spleen)
    • females on the CBA * DBA/2J (~90 x 106/spleen) have significantly more nucleated spleen cells CBA/HN females (~60 x 106/spleen)
  • decreased B cell number (MGI Ref ID J:81429)
    • spleens from females have ~40% immunoglobulin-bearing cells compared to ~50% in controls
    • numbers are greater in female mutants on CBA * C57BL/6 background (40%) than females on CBA/HN background (22%)

Btkxid/Y

        involves: CBA/HN * DBA/2N
  • hematopoietic system phenotype
  • abnormal spleen cellularity (MGI Ref ID J:81429)
    • number of nucleated spleen cells (~50 x 106/spleen) is significantly lower than in controls (~120 x 106/spleen)
  • decreased B cell number (MGI Ref ID J:81429)
    • spleens have only ~22% immunoglobulin-bearing cells compared to ~50% in controls
  • decreased B cell proliferation (MGI Ref ID J:81429)
    • cultured B cells show little response to B-cell mitogens LPS and poly I:C in comparison to wild type cells
    • proliferative response occurs slightly later and is much lower in males than in females
  • immune system phenotype
  • abnormal spleen cellularity (MGI Ref ID J:81429)
    • number of nucleated spleen cells (~50 x 106/spleen) is significantly lower than in controls (~120 x 106/spleen)
  • decreased B cell number (MGI Ref ID J:81429)
    • spleens have only ~22% immunoglobulin-bearing cells compared to ~50% in controls
  • decreased B cell proliferation (MGI Ref ID J:81429)
    • cultured B cells show little response to B-cell mitogens LPS and poly I:C in comparison to wild type cells
    • proliferative response occurs slightly later and is much lower in males than in females

Gene & Allele Details

Allele Symbol Btkxid
Allele Name X linked immune deficiency
Common Name(s) xid;
Strain of OriginCBA/HN
Gene Symbol and Name Btk, Bruton agammaglobulinemia tyrosine kinase
Chromosome X
Gene Common Name(s) AGMX1; AI528679; AT; ATK; BPK; Bruton's tyrosine kinase; IMD1; MGC126261; MGC126262; PSCTK1; X-linked immune deficiency; XLA; expressed sequence AI528679; xid;
Molecular Note The C to T transition point mutation at position 219 is predicted to change amino acid 28 from an arginine to a cysteine in the encoded protein. [MGI Ref ID J:13209]

Control Information

  Control
   000654 CBA/CaJ (approximate) The mating scheme, homozygote x hemizygote, does not generate a wild-type control. 000654 may be used as an approximate control whose degree of relationship to CBA/CaHN is uncertain.
 
  Considerations for Choosing Controls

Colony Maintenance

Diet Information LabDiet® 5K52/5K67

Related Strains

CBA Strains
001143   CBA/CaGnLeJ
000655   CBA/CaH-T(14;15)6Ca/J
000654   CBA/CaJ
000656   CBA/J
View CBA Strains     (4 strains)

Strains carrying other alleles of Btk
002536   B6;129S-Btktm1Wk/J
View Strains carrying other alleles of Btk     (1 strain)

Phenotypic Data

Mouse Phenome Database
Festing Inbred Strain Characteristics: CBA

Additional Web Information

Genetic Quality Control Annual Report

Animal Health Reports

Room Number           MP13

Research Applications

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

Btkxid related

Immunology and Inflammation Research
Immunodeficiency (B cell defects)

Mouse/Human Gene Homologs
Bruton agammaglobulinemia tyrosine kinase

Research Tools
Immunology and Inflammation Research (B cell deficiency)

References

Selected Reference(s)

Amsbaugh DF; Hansen CT; Prescott B; Stashak PW; Barthold DR; Baker PJ. 1972. Genetic control of the antibody response to type 3 pneumococcal polysaccharide in mice. I. Evidence that an X-linked gene plays a decisive role in determining responsiveness. J Exp Med 136(4):931-49. [PubMed: 4403476]  [MGI Ref ID J:109969]

Berning AK; Eicher EM; Paul WE; Scher I. 1980. Mapping of the X-linked immune deficiency mutation (xid) of CBA/N mice. J Immunol 124(4):1875-7. [PubMed: 7365241]  [MGI Ref ID J:6296]

Cancro MP; Sah AP; Levy SL; Allman DM; Schmidt MR; Woodland RT. 2001. xid mice reveal the interplay of homeostasis and Bruton's tyrosine kinase-mediated selection at multiple stages of B cell development. Int Immunol 13(12):1501-14. [PubMed: 11717191]  [MGI Ref ID J:109858]

Additional References

Price and Supply Information

Strain Name: CBA/CaHN-Btkxid/J
Stock Number: 001011

Price Details

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Supply Details

Standard SupplyLevel 2. Up to 100 mice. Larger quantities or custom orders arranged upon request.
Supply Notes Shipped at a specific age in weeks. Mice at a precise age in days, littermates and retired breeders are also available.
Strains that must be genotyped are not available until five to seven weeks of age.
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.

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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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