Strain Name:

B6;129S6-Del(17Abcg5-Abcg8)1Hobb/J

Stock Number:

004670

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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 B6;129S6-Abcg5/Abcg8tm1Hobb/J    (Changed: 12-SEP-14 )
B6;129S6-Abcg5/Abcg8tm1Hobb    (Changed: 15-DEC-04 )
Type Deletion;
Additional information on Mice with Chromosomal Aberrations.
Type Mutant Stock; Targeted Mutation;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Specieslaboratory mouse
 
Donating Investigator Helen H. Hobbs,   UT Southwestern Medical Center

Description
Mice that are homozygous for this targeted allele are viable, normal in size and do not display any gross physical or behavioral abnormalities. No gene product (mRNA or protein) is detected in liver or jejunum. A 2 to 3 fold increase in fractional absorption of dietary plant sterols results in plasma sitosterol levels that are elevated 30 fold compared to wildtype levels. Biliary cholesterol levels are low, as are plasma and liver cholesterol levels. Plasma and liver cholesterol levels increase rapidly (2.4 and 18 fold, respectively) following cholesterol feeding. This mutant mouse strain represents a model that may be useful in studies related to sitosterolemia and cholesterol homeostasis.

Development
The mouse Abcg5 and Abcg8 genes lie in a head-to-head configuration, separated by approximately 400 bp. A targeting vector containing a neomycin resistance gene driven by the mouse phosphoglycerate kinase promoter was used to disrupt Abcg5 exons 1 - 2, Abcg8 exons 1 - 3 and the sequence that lies between the two genes. The neomycin resistance gene is flanked by both loxP and FRT (FLP recombinase target sequence) sites. The construct was electroporated into 129S6/SvEv-derived SM1 embryonic stem (ES) cells. Correctly targeted ES cells were injected into C57BL/6J blastocysts. Chimeric mice were bred by the Donating Investigator to C57BL/6J once before making the line homozygous.

Control Information

  Control
   101045 B6129SF2/J (approximate)
 
  Considerations for Choosing Controls

Phenotype

Phenotype Information

View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Del(17Abcg5-Abcg8)1Hobb/Del(17Abcg5-Abcg8)1Hobb

        involves: 129S6/SvEvTac * C57BL/6
  • digestive/alimentary phenotype
  • abnormal intestinal lipid absorption
    • 2- to 3- fold increase in the fractional absorption of dietary plant sterols   (MGI Ref ID J:80679)
    • the fractional absorption of dietary cholesterol was not significantly different   (MGI Ref ID J:80679)
  • homeostasis/metabolism phenotype
  • abnormal cholesterol homeostasis
    • the cholesterol synthesis rate was significantly lower in the adrenal gland of the female mutant mice   (MGI Ref ID J:80679)
    • abnormal circulating cholesterol level
      • on 2% cholesterol diet, plasma and liver cholesterol levels increased significantly more than the increase in the wild-type   (MGI Ref ID J:80679)
      • decreased circulating cholesterol level
        • the plasma and liver cholesterol level were significantly lower than controls on normal diet   (MGI Ref ID J:80679)
  • abnormal circulating lipid level
    • approximately 30-fold increase in plasma sitosterol   (MGI Ref ID J:80679)
    • abnormal circulating cholesterol level
      • on 2% cholesterol diet, plasma and liver cholesterol levels increased significantly more than the increase in the wild-type   (MGI Ref ID J:80679)
      • decreased circulating cholesterol level
        • the plasma and liver cholesterol level were significantly lower than controls on normal diet   (MGI Ref ID J:80679)
  • abnormal intestinal lipid absorption
    • 2- to 3- fold increase in the fractional absorption of dietary plant sterols   (MGI Ref ID J:80679)
    • the fractional absorption of dietary cholesterol was not significantly different   (MGI Ref ID J:80679)
  • liver/biliary system phenotype
  • abnormal bile composition
    • bilary cholesterol concentrations were extremely low   (MGI Ref ID J:80679)
  • hematopoietic system phenotype
  • decreased platelet cell number
    • at 5 to 7 months of age, show marked macrothrombocytopenia   (MGI Ref ID J:157223)
  • increased mean platelet volume
    • at 5 to 7 months of age, show marked macrothrombocytopenia   (MGI Ref ID J:157223)
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
Vascular Defects
      platelet defect

Hematological Research
Platelet Defects
      thrombocytopenia

Internal/Organ Research
Liver Defects

Metabolism Research
Lipid Metabolism

Reproductive Biology Research
Fertility Defects

Research Tools
Cardiovascular Research
Internal/Organ Research
Metabolism Research

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Del(17Abcg5-Abcg8)1Hobb
Allele Name targeted mutation 1, Helen H Hobbs
Allele Type Targeted (Null/Knockout)
Common Name(s) Abcg5/Abcg8tm1Hobb; G5G8-;
Mutation Made By Liqing Yu,   Wake Forest Univ. School of Medicine
Strain of Origin129S6/SvEvTac
ES Cell Line NameSM1
ES Cell Line Strain129S6/SvEvTac
Gene Symbol and Name Del(17Abcg5-Abcg8)1Hobb, deletion, Chr 17, Helen H Hobbs 1
Chromosome 17
Molecular Note Both the Abcg5 and Abcg8 endogenous loci, which are oriented head to head and separated by approximately 400 bp, were disrupted by the integration of a single targeting construct. A neomycin selection cassette flanked by both loxP and frt sites replaced sequence extending from Abcg5 intron 2 into Abcg8 intron 3. The Walker A consensus sequences that make up a portion of the ATP binding sites in both genes were deleted. Northern and Western blot analyses indicated an absence of normal transcript and protein derived from both loci in homozygous mutant mice. [MGI Ref ID J:80679]

Genotyping

Genotyping Information

Genotyping Protocols

Abcg5/Abcg8tm1Hobb, Standard PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Yu L; Hammer RE; Li-Hawkins J; Von Bergmann K; Lutjohann D; Cohen JC; Hobbs HH. 2002. Disruption of Abcg5 and Abcg8 in mice reveals their crucial role in biliary cholesterol secretion. Proc Natl Acad Sci U S A 99(25):16237-42. [PubMed: 12444248]  [MGI Ref ID J:80679]

Additional References

Del(17Abcg5-Abcg8)1Hobb related

Calpe-Berdiel L; Rotllan N; Fievet C; Roig R; Blanco-Vaca F; Escola-Gil JC. 2008. Liver X receptor-mediated activation of reverse cholesterol transport from macrophages to feces in vivo requires ABCG5/G8. J Lipid Res 49(9):1904-11. [PubMed: 18509196]  [MGI Ref ID J:139767]

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]

Escola-Gil JC; Llaverias G; Julve J; Jauhiainen M; Mendez-Gonzalez J; Blanco-Vaca F. 2011. The cholesterol content of Western diets plays a major role in the paradoxical increase in high-density lipoprotein cholesterol and upregulates the macrophage reverse cholesterol transport pathway. Arterioscler Thromb Vasc Biol 31(11):2493-9. [PubMed: 21885848]  [MGI Ref ID J:191459]

McDaniel AL; Alger HM; Sawyer JK; Kelley KL; Kock ND; Brown JM; Temel RE; Rudel LL. 2013. Phytosterol Feeding Causes Toxicity in ABCG5/G8 Knockout Mice. Am J Pathol 182(4):1131-8. [PubMed: 23380580]  [MGI Ref ID J:194476]

Nguyen TM; Sawyer JK; Kelley KL; Davis MA; Kent CR; Rudel LL. 2012. ACAT2 and ABCG5/G8 are both required for efficient cholesterol absorption in mice: evidence from thoracic lymph duct cannulation. J Lipid Res 53(8):1598-609. [PubMed: 22669916]  [MGI Ref ID J:186555]

Su K; Sabeva NS; Liu J; Wang Y; Bhatnagar S; van der Westhuyzen DR; Graf GA. 2012. The ABCG5 ABCG8 sterol transporter opposes the development of fatty liver disease and loss of glycemic control independently of phytosterol accumulation. J Biol Chem 287(34):28564-75. [PubMed: 22715101]  [MGI Ref ID J:188929]

Tang W; Ma Y; Jia L; Ioannou YA; Davies JP; Yu L. 2009. Genetic inactivation of NPC1L1 protects against sitosterolemia in mice lacking ABCG5/ABCG8. J Lipid Res 50(2):293-300. [PubMed: 18796403]  [MGI Ref ID J:149007]

Wang J; Zhang DW; Lei Y; Xu F; Cohen JC; Hobbs HH; Xie XS. 2008. Purification and reconstitution of sterol transfer by native mouse ABCG5 and ABCG8. Biochemistry 47(18):5194-204. [PubMed: 18402465]  [MGI Ref ID J:134560]

Yamanashi Y; Takada T; Yoshikado T; Shoda J; Suzuki H. 2011. NPC2 regulates biliary cholesterol secretion via stimulation of ABCG5/G8-mediated cholesterol transport. Gastroenterology 140(5):1664-74. [PubMed: 21315718]  [MGI Ref ID J:192648]

Yang C; Yu L; Li W; Xu F; Cohen JC; Hobbs HH. 2004. Disruption of cholesterol homeostasis by plant sterols. J Clin Invest 114(6):813-22. [PubMed: 15372105]  [MGI Ref ID J:92860]

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]

Yu L; von Bergmann K; Lutjohann D; Hobbs HH; Cohen JC. 2005. Ezetimibe normalizes metabolic defects in mice lacking ABCG5 and ABCG8. J Lipid Res 46(8):1739-44. [PubMed: 15930515]  [MGI Ref ID J:100478]

Yu L; von Bergmann K; Lutjohann D; Hobbs HH; Cohen JC. 2004. Selective sterol accumulation in ABCG5/ABCG8-deficient mice. J Lipid Res 45(2):301-7. [PubMed: 14657202]  [MGI Ref ID J:121052]

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 & HusbandryWhen maintaining a live colony, these mice are bred mating heterozygote females to homozygote males. Homozygous breeder pair matings have been found to be unproductive by The Jackson Laboratory colony managers (February 3, 2005).

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.

Frozen Products

Price (US dollars $)
Frozen Embryo $1650.00

Standard Supply

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

Supply Notes

  • Cryopreserved Embryos
    Available to most shipping destinations1
    This strain is also available as cryopreserved embryos2. Orders for cryopreserved embryos may be placed with our Customer Service Department. Experienced technicians at The Jackson Laboratory have recovered frozen embryos of this strain successfully. We will provide you enough embryos to perform two embryo transfers. The Jackson Laboratory does not guarantee successful recovery at your facility. For complete information on purchasing embryos, please visit our Cryopreserved Embryos web page.

    1 Shipments cannot be made to Australia due to Australian government import restrictions.
    2 Embryos for most strains are cryopreserved at the two cell stage while some strains are cryopreserved at the eight cell stage. If this information is important to you, please contact Customer Service.
  • 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.

Frozen Products

Price (US dollars $)
Frozen Embryo $2145.00

Standard Supply

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

Supply Notes

  • Cryopreserved Embryos
    Available to most shipping destinations1
    This strain is also available as cryopreserved embryos2. Orders for cryopreserved embryos may be placed with our Customer Service Department. Experienced technicians at The Jackson Laboratory have recovered frozen embryos of this strain successfully. We will provide you enough embryos to perform two embryo transfers. The Jackson Laboratory does not guarantee successful recovery at your facility. For complete information on purchasing embryos, please visit our Cryopreserved Embryos web page.

    1 Shipments cannot be made to Australia due to Australian government import restrictions.
    2 Embryos for most strains are cryopreserved at the two cell stage while some strains are cryopreserved at the eight cell stage. If this information is important to you, please contact Customer Service.
  • 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
   101045 B6129SF2/J (approximate)
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

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