Strain Name:

B6SJL-Tg(ABCG5/ABCG8)14-2Hobb/J

Stock Number:

004583

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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

Former Names B6SJL-Tg(ABCG5/ABCG8)14-2Hobb    (Changed: 15-DEC-04 )
Type Mutant Stock; Transgenic;
Additional information on Genetically Engineered and Mutant Mice.
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Mating System+/+ sibling x Hemizygote         (Female x Male)   25-JUN-08
Specieslaboratory mouse
 
Donating Investigator Helen H. Hobbs,   UT Southwestern Medical Center

Description
These transgenic mice over-express the human ABCG5 and ABCG8 genes under the direction of their endogenous regulatory sequences. Copy number of the transgene was estimated using Southern blot analysis to compare transgenic mouse genomic DNA to human genomic DNA. These transgenic mice have approximately ten copies of the transgene. Northern blot analysis revealed the human transgene is expressed in the liver and small intestine. RT-PCR showed trace levels of transgene transcript in ovary tissue. Absorption of dietary cholesterol in transgenic mice is reduced by 50%. Mean fasting plasma cholesterol levels are significantly lower in female transgenic mice. Excretion of neutral sterols in feces is elevated to three times higher above normal in male transgenic mice and six times higher in female transgenic mice. Bile from transgenic animals is opaque as compared to the clear bile of wildtype animals. Cholesterol in the bile is elevated to levels five times higher than normal in male transgenic mice and seven times higher in female transgenic mice. The phospholipid level in the bile of male transgenic mice is slightly increased. When fed a high-cholesterol diet, transgenic mice did not exhibit any significant change in hepatic cholesterol levels. Genes encoding proteins involved in cholesterol synthesis are up regulated. Mice homozygous for the transgenic insert are viable and fertile. This mutant mouse strain may be useful in studies of cholesterol trafficking and cholesterol gallstone disease.

Development
A transgenic construct containing a 90kb of sequence including the entire coding regions of the human ABCG5 and ABCG8 genes, approximately 30Kb of sequence flanking the 3' end of ABCG5, 6Kb of sequence flanking the 3" end of ABCG8 and sequence encoding exons 6 to 13 of putative gene CGI-60 was injected into fertilized C57BL/6J X SJL F2 (Stock # 100012) mouse eggs. Founder animals were bred to C57BL/6J X SJL F1 mice.

Control Information

  Control
   Noncarrier
 
  Considerations for Choosing Controls

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- Potential model based on transgenic expression of an ortholog of a human gene that is associated with this disease. Phenotypic similarity to the human disease has not been tested.
Gallbladder Disease 4; GBD4   (ABCG8)
Sitosterolemia   (ABCG5)
Sitosterolemia   (ABCG8)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Tg(ABCG5/ABCG8)14-2Hobb/0

        involves: C57BL/6J * SJL
  • homeostasis/metabolism phenotype
  • abnormal cholesterol homeostasis
    • cholesterol synthesis is increased 3-fold in the female duodenum, decreased in male jejunum and ileum, and decreased in the female ileum compared to in wild-type mice   (MGI Ref ID J:78963)
    • however, fatty acid synthesis is normal   (MGI Ref ID J:78963)
    • decreased circulating cholesterol level
      • in fasting female mice   (MGI Ref ID J:78963)
      • decreased circulating HDL cholesterol level
        • in fasting female mice   (MGI Ref ID J:78963)
    • increased cholesterol level
      • bile cholesterol levels are increased 5-fold in male mice and 7-fold in female mice compared to in wild-type mice   (MGI Ref ID J:78963)
  • abnormal circulating plant sterol level
    • plasma sitosterol levels are decreased 64% and 79% in males and females, respectively, compared to in wild-type mice   (MGI Ref ID J:78963)
    • plasma levels of plant sterol and campesterol are reduced compared to in wild-type mice   (MGI Ref ID J:78963)
    • however, the plasma levels of lathosterol are normal   (MGI Ref ID J:78963)
    • plasma sitosterol and campesterol levels are decreased compared to in wild-type mice   (MGI Ref ID J:100491)
  • decreased intestinal cholesterol absorption
  • liver/biliary system phenotype
  • abnormal bile composition
    • bile cholesterol levels are increased 5-fold in male mice and 7-fold in female mice compared to in wild-type mice   (MGI Ref ID J:78963)
    • bile cholesterol levels are increased compared to in wild-type mice   (MGI Ref ID J:100491)
  • decreased susceptibility to hepatic steatosis
    • when fed a high fat diet   (MGI Ref ID J:78963)
  • digestive/alimentary phenotype
  • abnormal feces composition
    • feces sterol levels are 3-fold higher in males and 6-fold higher in females compared to in wild-type mice   (MGI Ref ID J:78963)
    • fecal sterol excretion is increased 2- to 3-fold compared to in wild-type mice   (MGI Ref ID J:100491)
    • steatorrhea
      • in female but not male mice   (MGI Ref ID J:78963)
  • abnormal intestinal absorption
    • campesterol and sitosterol absorption are decreased compared to in wild-type mice   (MGI Ref ID J:100491)
    • decreased intestinal cholesterol absorption
View Research Applications

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

Cardiovascular Research
Hypercholesterolemia
Hypocholesterolemia
Metabolic Syndrome
Other
      altered fat metabolism
      altered lipoprotein profile

Cell Biology Research
Channel and Transporter Defects

Internal/Organ Research
Liver Defects
Other

Research Tools
Cardiovascular Research
Internal/Organ Research
Metabolism Research

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Tg(ABCG5/ABCG8)14-2Hobb
Allele Name transgene insertion 14-2, Helen H Hobbs
Allele Type Transgenic (Inserted expressed sequence)
Common Name(s) ABCG5/ABCG8; G5G8Tg; hG5G8Tg;
Mutation Made By Liqing Yu,   Wake Forest Univ. School of Medicine
Strain of Origin(C57BL/6J x SJL)F2
Expressed Gene ABCG5, ATP-binding cassette, sub-family G (WHITE), member 5, human
Expressed Gene ABCG8, ATP-binding cassette, sub-family G (WHITE), member 8, human
Promoter ABCG5, ATP-binding cassette, sub-family G (WHITE), member 5, human
Promoter ABCG8, ATP-binding cassette, sub-family G (WHITE), member 8, human
Molecular Note The transgene contains a 90kb of sequence including the entire coding regions of the human ABCG5 and ABCG8 genes, approximately 30Kb of sequence flanking the 3' end of ABCG5, 6Kb of sequence flanking the 3' end of ABCG8 and sequence encoding exons 6 to 13 of a putative gene called CGI-60. Estimated transgene copy number is ~14 for this line. Northern blot analysis revealed the human transgene is expressed in the liver and small intestine. RT-PCR showed trace levels of transgene transcript in ovary tissue. [MGI Ref ID J:78963]
 
 

Genotyping

Genotyping Information

Genotyping Protocols

Tg(ABCG5/ABCG8)14-2Hobb, Standard PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Yu L; Li-Hawkins J; Hammer RE; Berge KE; Horton JD; Cohen JC; Hobbs HH. 2002. Overexpression of ABCG5 and ABCG8 promotes biliary cholesterol secretion and reduces fractional absorption of dietary cholesterol. J Clin Invest 110(5):671-80. [PubMed: 12208868]  [MGI Ref ID J:78963]

Additional References

Tg(ABCG5/ABCG8)14-2Hobb related

Chase TH; Lyons BL; Bronson RT; Foreman O; Donahue LR; Burzenski LM; Gott B; Lane P; Harris B; Ceglarek U; Thiery J; Wittenburg H; Thon JN; Italiano JE Jr; Johnson KR; Shultz LD. 2010. The mouse mutation 'thrombocytopenia and cardiomyopathy' (trac) disrupts Abcg5: a spontaneous single gene model for human hereditary phytosterolemia/sitosterolemia. Blood 115(6):1267-76. [PubMed: 19846887]  [MGI Ref ID J:157223]

Langheim S; Yu L; von Bergmann K; Lutjohann D; Xu F; Hobbs HH; Cohen JC. 2005. ABCG5 and ABCG8 require MDR2 for secretion of cholesterol into bile. J Lipid Res 46(8):1732-8. [PubMed: 15930516]  [MGI Ref ID J:100491]

Yu L; Gupta S; Xu F; Liverman AD; Moschetta A; Mangelsdorf DJ; Repa JJ; Hobbs HH; Cohen JC. 2005. Expression of ABCG5 and ABCG8 is required for regulation of biliary cholesterol secretion. J Biol Chem 280(10):8742-7. [PubMed: 15611112]  [MGI Ref ID J:128570]

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 & HusbandryA transgenic construct containing the entire coding regions of the human ABCG5 and ABCG8 genes was used to create transgenic animals on a B6SJL background. The strain is maintained as a hemizygote.
Mating System+/+ sibling x Hemizygote         (Female x Male)   25-JUN-08

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
   Noncarrier
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

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