Strain Name:

B6.Cg-Spta1sph-2Bc/BrkJ

Stock Number:

001185

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Availability:

Cryopreserved - Ready for recovery

Description

The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.

Strain Information

Former Names C57BL/6J-Spna1sph-2Bc/J    (Changed: 22-MAR-10 )
Type Congenic; Mutant Strain; Spontaneous Mutation;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Additional information on Congenic nomenclature.
Specieslaboratory mouse
GenerationN27p
Generation Definitions

Related Strains

Strains carrying   Spta1sph-2Bc allele
001184   WB.Cg-Spta1sph-2Bc/BrkJ
View Strains carrying   Spta1sph-2Bc     (1 strain)

Strains carrying other alleles of Spta1
000450   B6.C3-Spta1sph/BrkJ
003815   B6.CcS3-Spta1sph-Dem/BrkJ
006210   B6.Cg-Spta1ihj/LlpJ
000446   B6.D1-Spta1sph-ha/BrkJ
007875   B6.NOD(Cg)-Spta1sph-3J/LlpJ
000454   WB.C3-Spta1sph/BrkJ
003816   WB.CcS3-Spta1sph-Dem/BrkJ
000451   WB.D1-Spta1sph-ha/BrkJ
View Strains carrying other alleles of Spta1     (8 strains)

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- Model with phenotypic similarity to human disease where etiologies involve orthologs. Human genes are associated with this disease. Orthologs of those genes appear in the mouse genotype(s).
Spherocytosis, Type 3; SPH3
- Model with phenotypic similarity to human disease where etiologies are distinct. Human genes are associated with this disease. Orthologs of these genes do not appear in the mouse genotype(s).
Spherocytosis, Type 1; SPH1
- Potential model based on gene homology relationships. Phenotypic similarity to the human disease has not been tested.
Elliptocytosis 2; EL2   (SPTA1)
Pyropoikilocytosis, Hereditary; HPP   (SPTA1)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

The following phenotype information is associated with a similar, but not exact match to this JAX® Mice strain.

Spta1sph-2Bc/Spta1sph-2Bc

        involves: SELH
  • mortality/aging
  • partial postnatal lethality
    • about one-fourth survive to weaning and one-third of these to 6 months   (MGI Ref ID J:7048)
  • premature death   (MGI Ref ID J:7048)
  • hematopoietic system phenotype
  • abnormal erythrocyte morphology
    • absence of alpha-spectrin in the red cell membrane skeleton   (MGI Ref ID J:7501)
    • abnormal forms of red blood cells include spherocytes, schistocytes, anisocytes, poikilocytes, erythroblasts, stipple cells, and elliptocytes   (MGI Ref ID J:7048)
    • anisocytosis   (MGI Ref ID J:7048)
    • decreased hematocrit
      • hematocrit is reduced by half   (MGI Ref ID J:7048)
    • decreased hemoglobin content
      • hemoglobin level is reduced by more than half   (MGI Ref ID J:7048)
    • hemolytic anemia
      • spherocytic, hemolytic anemia   (MGI Ref ID J:7048)
    • poikilocytosis   (MGI Ref ID J:7048)
      • schistocytosis   (MGI Ref ID J:7048)
    • spherocytosis   (MGI Ref ID J:7048)
  • abnormal erythrocyte osmotic lysis
    • osmotic fragility is increased in red blood cells; red blood cells hemolyse at higher NaCl concentrations than controls   (MGI Ref ID J:7048)
  • abnormal spleen morphology
    • spleens are deep purple instead of red   (MGI Ref ID J:7048)
    • enlarged spleen
      • splenomegaly is seen in adults   (MGI Ref ID J:7048)
  • erythroblastosis   (MGI Ref ID J:7048)
  • extramedullary hematopoiesis
    • extramedullary hematopoiesis in the liver   (MGI Ref ID J:7048)
  • increased bone marrow cell number
    • erythroid hyperplasia in the bone marrow   (MGI Ref ID J:7048)
  • increased leukocyte cell number
    • increased in white blood cell count   (MGI Ref ID J:7048)
  • reticulocytosis
    • increase in reticulocyte count (90% vs. 5-10% in controls)   (MGI Ref ID J:7048)
  • liver/biliary system phenotype
  • jaundice
    • jaundice appears after birth   (MGI Ref ID J:7048)
  • homeostasis/metabolism phenotype
  • increased circulating bilirubin level
    • hyperbilirubinemia is seen as orange deposits in the liver and intestines   (MGI Ref ID J:7048)
  • immune system phenotype
  • abnormal spleen morphology
    • spleens are deep purple instead of red   (MGI Ref ID J:7048)
    • enlarged spleen
      • splenomegaly is seen in adults   (MGI Ref ID J:7048)
  • increased leukocyte cell number
    • increased in white blood cell count   (MGI Ref ID J:7048)
  • integument phenotype
  • pallor
    • mice are pale at birth   (MGI Ref ID J:7048)

Spta1sph-2Bc/Spta1sph-2Bc

        (WB.Cg-Spta1sph-2Bc/Brk x B6.Cg-Spta1sph-2Bc/Brk)F1
  • hematopoietic system phenotype
  • abnormal erythrocyte morphology
    • cell surface expression of phosphatidylserine is higher than in wild-type controls but not as high as in other hemolytic anemia mutants, with 4.9% of erythrocytes straining positive for phosphatidylserine versus 1.6% in wild-type controls   (MGI Ref ID J:62355)
    • abnormal red blood cell deformability
      • red blood cells exhibit a profound decrease in surface area and marked increase in osmotic fragility   (MGI Ref ID J:81125)
    • microcytosis
      • the percentage of erythroid cells that are microcytes is larger than in wild-type controls   (MGI Ref ID J:62355)
  • homeostasis/metabolism phenotype
  • thrombosis
    • 100% of homozygous adults display cardiac thrombi   (MGI Ref ID J:62355)

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Spta1sph-2Bc
Allele Name spherocytosis 2 British Columbia
Allele Type Spontaneous
Common Name(s) sph2Bc;
Strain of Originmixed
Gene Symbol and Name Spta1, spectrin alpha, erythrocytic 1
Chromosome 1
Gene Common Name(s) AF093576; AI451697; EL2; HPP; HS3; SPH3; SPTA; Spna-1; Spna1; erythroid; expressed sequence AF093576; expressed sequence AI451697; ha; hemolytic anemia; iasi hereditary jaundice; ihj; spectrin alpha 1; sph; spherocytosis;
General Note This allele arose from a mixed SELH stock at the University of British Columbia in 1983.
Molecular Note The mutation in the sph-BC mouse was identified as a G to T in transversion in the first nucleotide of intron 41. This mutation causes a splicing defect that results in the skipping of exon 41 causing the deletion of 54 amino acids as well as a frameshift and a premature stop codon. [MGI Ref ID J:81125]

Genotyping

Genotyping Information


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Additional References

Spta1sph-2Bc related

Bodine DM 4th; Birkenmeier CS; Barker JE. 1984. Spectrin deficient inherited hemolytic anemias in the mouse: characterization by spectrin synthesis and mRNA activity in reticulocytes. Cell 37(3):721-9. [PubMed: 6234993]  [MGI Ref ID J:7501]

Juriloff DM. 1983. Spna1<sph-2Bc> - spherocytosis-2Bc Mouse News Lett 68:59.  [MGI Ref ID J:64113]

Peters LL; Barker JE. 2001. Spontaneous and targeted mutations in erthrocyte membrane skeleton genes: mouse models of heredity spherocytosis. In: Hematopoiesis A Developmental Approach. Oxford University Press.  [MGI Ref ID J:88022]

Robledo RF; Lambert AJ; Birkenmeier CS; Cirlan MV; Cirlan AF; Campagna DR; Lux SE; Peters LL. 2010. Analysis of novel sph (spherocytosis) alleles in mice reveals allele-specific loss of band 3 and adducin in {alpha}-spectrin-deficient red cells. Blood 115(9):1804-14. [PubMed: 20056793]  [MGI Ref ID J:157766]

Unger AE; Harris MJ; Bernstein SE; Falcone JC; Lux SE. 1983. Hemolytic anemia in the mouse. Report of a new mutation and clarification of its genetics. J Hered 74(2):88-92. [PubMed: 6841965]  [MGI Ref ID J:7048]

Wandersee NJ; Birkenmeier CS; Bodine DM; Mohandas N; Barker JE. 2003. Mutations in the murine erythroid alpha -spectrin gene alter spectrin mRNA and protein levels and spectrin incorporation into the red blood cell membrane skeleton. Blood 101(1):325-30. [PubMed: 12393645]  [MGI Ref ID J:81125]

Wandersee NJ; Tait JF; Barker JE. 2000. Erythroid phosphatidyl serine exposure is not predictive of thrombotic risk in mice with hemolytic anemia Blood Cells Mol Dis 26(1):75-83. [PubMed: 10772878]  [MGI Ref ID J:62355]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, G200.

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls


Pricing for USA, Canada and Mexico shipping destinations View International Pricing

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $3175.00
Animals Provided

At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Supply Notes

  • Cryorecovery of Strains Needing Progeny Testing
    At least two untested males and two untested females (two pairs) will be recovered (eight or more mice is typical). The total number of animals provided, their gender and genotype will vary. Untested animals typically are available to ship between 13 and 16 weeks from the date of your order. If the first recovery attempt is unsuccessful, a second recovery will be done, extending the overall recovery time to approximately 25 weeks. Progeny testing is required to identify the genotype of mice of this strain, as a genotyping assay is not available. This type of testing involves breeding the recovered animals and assessing the phenotype of the offspring in order to identify animals carrying the mutation of interest. We can perform the progeny testing for you as a service or we can ship all recovered animals to you for progeny testing at your facility. If you perform the progeny testing, there is NO guarantee that a carrier will be identified. If we perform progeny testing as a service, additional breeding time will be required. In this case, when a male and female (one pair) are identified that carry the mutation, they and their offspring will be shipped. Delivery time for strains requiring progeny testing often exceeds 25 weeks and may take 12 months or more due to the difficulties in breeding some strains. The progeny testing cost is in addition to the recovery cost and is based on the number of boxes used and the time taken to produce the mice identified as carrying the mutation.
    Please note that identified pairs may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation of the strain. Mating schemes are sometimes modified for successful cryopreservation.

    Please contact Customer Service for more information on the cost of progeny testing for a strain: Tel: 1-800-422-6423 or 1-207-288-5845 (from any location). The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice.
    Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).

Pricing for International shipping destinations View USA Canada and Mexico Pricing

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $4127.50
Animals Provided

At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Supply Notes

  • Cryorecovery of Strains Needing Progeny Testing
    At least two untested males and two untested females (two pairs) will be recovered (eight or more mice is typical). The total number of animals provided, their gender and genotype will vary. Untested animals typically are available to ship between 13 and 16 weeks from the date of your order. If the first recovery attempt is unsuccessful, a second recovery will be done, extending the overall recovery time to approximately 25 weeks. Progeny testing is required to identify the genotype of mice of this strain, as a genotyping assay is not available. This type of testing involves breeding the recovered animals and assessing the phenotype of the offspring in order to identify animals carrying the mutation of interest. We can perform the progeny testing for you as a service or we can ship all recovered animals to you for progeny testing at your facility. If you perform the progeny testing, there is NO guarantee that a carrier will be identified. If we perform progeny testing as a service, additional breeding time will be required. In this case, when a male and female (one pair) are identified that carry the mutation, they and their offspring will be shipped. Delivery time for strains requiring progeny testing often exceeds 25 weeks and may take 12 months or more due to the difficulties in breeding some strains. The progeny testing cost is in addition to the recovery cost and is based on the number of boxes used and the time taken to produce the mice identified as carrying the mutation.
    Please note that identified pairs may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation of the strain. Mating schemes are sometimes modified for successful cryopreservation.

    Please contact Customer Service for more information on the cost of progeny testing for a strain: Tel: 1-800-422-6423 or 1-207-288-5845 (from any location). The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice.
    Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).

View USA Canada and Mexico Pricing View International Pricing

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

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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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