Strain Name:

B6.129S6-Abcb11tm1Wng/J

Stock Number:

004125

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Availability:

Cryopreserved - Ready for recovery

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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

Type Congenic; Mutant Strain; Targeted Mutation;
Additional information on Genetically Engineered and Mutant Mice.
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Additional information on Congenic nomenclature.
Specieslaboratory mouse
 
Donating Investigator Victor Ling,   British Columbia Cancer Agency

Description
Mice that are homozygous null for the Abcb11 gene suffer from lowered rates of viability and fertility. No Abcb11 gene product (mRNA or protein) is detected in liver tissue. Homozygotes display growth retardation with body weights being 80% that of wildtype littermates at weaning. Lower body weights persist throughout life. Ultrastructural changes are noted in the hepatic canaliculi (lumen dilation, microvilli loss, and accumulation of biliary material). Hepatocytes exhibit increased numbers of peroxisomes, lysosomes and lipid droplets with a concomitant decrease in stored glycogen. Although average bile flow is not significantly reduced, secretion of major hydrophobic bile salts is clearly impaired. An increase in the secretion of tetra-hydroxylated bile acids, cholesterol and phospholipids is observed. These mice provide a model for studying intrahepatic cholestasis and the mechanisims associated with lipid homeostasis.

Development
A targeting vector containing a neomycin resistance cassette in an antisense orientation with respect to the target gene was used to disrupt the region of the Abcb11 gene encoding amino acids 454-478. Three missense mutations and a premature stop codon at amino acid 454 were also introduced. The construct was electroporated into 129S6/SvEvTac-derived TL-1 embryonic stem (ES) cells. Correctly targeted ES cells were injected into C57BL/6J blastocysts. The resulting chimeric mice were backcrossed to C57BL/6LJ animals.

Control Information

  Control
   Wild-type male from the colony
   000664 C57BL/6J
 
  Considerations for Choosing Controls

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- Potential model based on gene homology relationships. Phenotypic similarity to the human disease has not been tested.
Cholestasis, Benign Recurrent Intrahepatic, 2; BRIC2   (ABCB11)
Cholestasis, Progressive Familial Intrahepatic, 2; PFIC2   (ABCB11)
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.

Abcb11tm1Wng/Abcb11tm1Wng

        involves: 129S6/SvEvTac * C57BL/6J
  • growth/size/body phenotype
  • decreased body weight
    • body weight is about 20% lower than in wild-type at weaning and lower weight is seen throughout life   (MGI Ref ID J:67548)
  • postnatal growth retardation   (MGI Ref ID J:67548)
  • homeostasis/metabolism phenotype
  • abnormal bile salt level
    • total concentration of biliary bile salts is reduced more than 4-fold   (MGI Ref ID J:67548)
    • increased bile salt level
      • total bile salt concentrations in the liver and plasma are elevated 4- and 5.8-fold, respectively   (MGI Ref ID J:67548)
      • mutants exhibit increased amounts of tetra-hydroxylated bile acids   (MGI Ref ID J:67548)
  • abnormal phospholipid level
    • 2.5-fold increase in biliary phospholipid concentration   (MGI Ref ID J:67548)
  • decreased glycogen level
    • in the liver   (MGI Ref ID J:67548)
  • increased cholesterol level
    • 7-fold increase in biliary cholesterol concentration   (MGI Ref ID J:67548)
  • liver/biliary system phenotype
  • abnormal bile canaliculus morphology
    • dilation of canalicular lumens, partial or complete loss of microvilli, and retention of biliary material   (MGI Ref ID J:67548)
  • abnormal bile secretion
    • impaired secretion of major hydrophobic bile salts, such as taurine and glycine-conjugated cholic acid, however secretion of the more hydrophilic muricholic acids is unaffected   (MGI Ref ID J:67548)
  • abnormal hepatocyte morphology
    • increase in peroxisomes, lysosomes, and lipid droplets   (MGI Ref ID J:67548)
  • enlarged liver
    • liver is enlarged to 10.1% of the body weight   (MGI Ref ID J:67548)
  • intrahepatic cholestasis
    • nonprogressive but persistent intrahepatic cholestasis   (MGI Ref ID J:67548)
View Research Applications

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

Abcb11tm1Wng related

Internal/Organ Research
Liver Defects

Research Tools
Internal/Organ Research
Metabolism Research

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Abcb11tm1Wng
Allele Name targeted mutation 1, Renxue Wang
Allele Type Targeted (Null/Knockout)
Common Name(s) spgp-;
Mutation Made By Renxue Wang,   British Columbia Cancer Agency
Strain of Origin129S6/SvEvTac
ES Cell Line NameTL1/TL-1
ES Cell Line Strain129S6/SvEvTac
Gene Symbol and Name Abcb11, ATP-binding cassette, sub-family B (MDR/TAP), member 11
Chromosome 2
Gene Common Name(s) ABC16; BRIC2; BSEP; Bsep; Lith1; PFIC-2; PFIC2; PGY4; SPGP; bile salt export pump; sister of P-glycoprotein;
Molecular Note The N-terminal ATP-binding domain, corresponding to amino acids 454-478, was deleted and replaced with a neo cassette. The construct introduced three missense mutations and a premature stop codon at position 454. Western analysis showed no detectable protein in homozygous mice. [MGI Ref ID J:67548]

Genotyping

Genotyping Information

Genotyping Protocols

Abcb11tm1Wng, Standard PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Wang R; Salem M; Yousef IM; Tuchweber B; Lam P; Childs SJ; Helgason CD; Ackerley C; Phillips MJ; Ling V. 2001. Targeted inactivation of sister of P-glycoprotein gene (spgp) in mice results in nonprogressive but persistent intrahepatic cholestasis. Proc Natl Acad Sci U S A 98(4):2011-6. [PubMed: 11172067]  [MGI Ref ID J:67548]

Additional References

Perwaiz S; Forrest D; Mignault D; Tuchweber B; Phillip MJ; Wang R; Ling V; Yousef IM. 2003. Appearance of atypical 3 alpha,6 beta,7 beta,12 alpha-tetrahydroxy-5 beta-cholan-24-oic acid in spgp knockout mice. J Lipid Res 44(3):494-502. [PubMed: 12562825]  [MGI Ref ID J:82194]

Abcb11tm1Wng related

Lam P; Wang R; Ling V. 2005. Bile acid transport in sister of P-glycoprotein (ABCB11) knockout mice. Biochemistry 44(37):12598-605. [PubMed: 16156672]  [MGI Ref ID J:101161]

Perwaiz S; Forrest D; Mignault D; Tuchweber B; Phillip MJ; Wang R; Ling V; Yousef IM. 2003. Appearance of atypical 3 alpha,6 beta,7 beta,12 alpha-tetrahydroxy-5 beta-cholan-24-oic acid in spgp knockout mice. J Lipid Res 44(3):494-502. [PubMed: 12562825]  [MGI Ref ID J:82194]

Wang R; Lam P; Liu L; Forrest D; Yousef IM; Mignault D; Phillips MJ; Ling V. 2003. Severe cholestasis induced by cholic acid feeding in knockout mice of sister of P-glycoprotein. Hepatology 38(6):1489-99. [PubMed: 14647060]  [MGI Ref ID J:105993]

Wang R; Liu L; Sheps JA; Forrest D; Hofmann AF; Hagey LR; Ling V. 2013. Defective canalicular transport and toxicity of dietary ursodeoxycholic acid in the abcb11-/- mouse: transport and gene expression studies. Am J Physiol Gastrointest Liver Physiol 305(4):G286-94. [PubMed: 23764895]  [MGI Ref ID J:205077]

Zhang Y; Li F; Patterson AD; Wang Y; Krausz KW; Neale G; Thomas S; Nachagari D; Vogel P; Vore M; Gonzalez FJ; Schuetz JD. 2012. Abcb11 deficiency induces cholestasis coupled to impaired beta-fatty acid oxidation in mice. J Biol Chem 287(29):24784-94. [PubMed: 22619174]  [MGI Ref ID J:188866]

Health & husbandry

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.

Health & Colony Maintenance Information

Animal Health Reports

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

Colony Maintenance

Breeding & HusbandryThis strain originated on a B6;129S6 background and has been backcrossed to C57BL/6J for at least eleven generations (7/01).

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* $2525.00
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 - Standard.
    Progeny testing is not required.

    The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 10 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. 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* $3283.00
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 - Standard.
    Progeny testing is not required.

    The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 10 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. 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.

Control Information

  Control
   Wild-type male from the colony
   000664 C57BL/6J
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

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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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