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| Mice that are homozygous for this Abcg8 (ATP-binding cassette, sub-family G (WHITE), member 8) targeted mutation exhibit increased plasma and tissue levels of sitosterol and campesterol (plant sterols) and infertility if not maintained on a plant sterol free diet. This mutant mouse strain may be useful in studies of sitosterolemia and phytosterolemia. | |||||||||||||||
- Description
- Disease & phenotype
- Genes & alleles
- Genotyping
- Health & care
- References
- Pricing & purchasing
- Terms of use
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 B6.129P2-Abcg8tm1Elk/J (Changed: 19-DEC-08 ) Type Congenic; Targeted Mutation; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Additional information on Congenic nomenclature. Species laboratory mouse Donating Investigator Shailendra B Patel, Medical College of Wisconsin Description
Mice that are heterozygous for the targeted mutation are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. Female homozygotes are infertile, and male homozygotes exhibit rare infertility, if not maintained on a plant sterol free diet or with drug treatment to block uptake of plant sterols. No gene product (mRNA) is detected by Northern blot and RT-PCR analysis of total hepatic RNA from homozygous animals. Homozygotes have increased plasma and tissue levels of sitosterol and campesterol (plant sterols) with impaired cholesterol secretion in bile. Expression of Abcg5 (ATP-binding cassette, sub-family G (WHITE), member 5) is unchanged in homozygotes. Liver cholesterol levels in homozygotes is decreased by approximately 50%. Serum cholesterol levels are decreased by 52% in homozygotes and 26% in heterozygotes. Heterozygotes exhibit an intermediate phenotype with decreased biliary sterol secretion but do not become sitosterolemic. This mutant mouse strain may be useful in studies of sitosterolemia and phytosterolemia.Development
A targeting vector containing neomycin resistance and herpes simplex virus thymidine kinase genes was used to disrupt parts of exons 3 and 4 and normal splicing. The construct was electroporated into 129P2/OlaHsd derived E14TG2a embryonic stem (ES) cells (as reported by the Donating Investigator). Correctly targeted ES cells were injected into C57BL/6 blastocysts. The resulting chimeric animals were crossed to C57BL/6J mice. Heterozygotes were intercrossed to generate homozygotes and the mice were then backcrossed to C57BL/6J for 17 generations.
Control Information
| Control | ||
|---|---|---|
| Wild-type from the colony | ||
| 000664 C57BL/6J | ||
| Considerations for Choosing Controls | ||
Related Strains
Strains carrying other alleles of Abcg8
004670 B6;129S6-Abcg5/Abcg8tm1Hobb/J View Strains carrying other alleles of Abcg8 (1 strain)
Phenotype
Phenotype Information
- Potential model based on gene homology relationships. Phenotypic similarity to the human disease has not been tested. Gallbladder Disease 4; GBD4 (ABCG8)
Sitosterolemia (ABCG8)
assigned by genotype
The following phenotype information may relate to a genetic background differing from this JAX® Mice strain.
Abcg8tm1Elk/Abcg8+
involves: 129S6/SvEvTac * C57BL/6J
- homeostasis/metabolism phenotype
- decreased circulating cholesterol level
- plasma cholesterol level was decreased by 26% (MGI Ref ID J:94759)
- liver/biliary system phenotype
- abnormal hepatobiliary system physiology
- under conditions that force biliary sterol secretion, cholesterol secretion into bile was reduced relative to wild-type (MGI Ref ID J:94759)
This mouse can be used to support research in many areas including:
Cardiovascular Research
Hypocholesterolemia
Metabolic Syndrome
Other
altered fat metabolism
altered lipoprotein profile
Metabolism Research
Lipid Metabolism
Research Tools
Metabolism Research
Genes & Alleles
Gene & Allele Information provided by MGI
| Allele Symbol | Abcg8tm1Elk | ||
|---|---|---|---|
| Allele Name | targeted mutation 1, Eric L Klett | ||
| Allele Type | Targeted (knock-out) | ||
| Common Name(s) | Abcg8/sterolin2; Abcg8-; | ||
| Mutation Made By | Shailendra Patel, Medical College of Wisconsin | ||
| Strain of Origin | 129S6/SvEvTac | ||
| ES Cell Line Strain | 129 | ||
| Gene Symbol and Name | Abcg8, ATP-binding cassette, sub-family G (WHITE), member 8 | ||
| Chromosome | 17 | ||
| Gene Common Name(s) | 1300003C16Rik; AI114946; GBD4; RIKEN cDNA 1300003C16 gene; STSL; Sterolin-2; expressed sequence AI114946; | ||
| Molecular Note | Part of exon 3 through part of exon 4 was replaced by a neomycin resistance cassette resulting in disruption of the ABC Walker A motif. No transcription was detected by Northern blot analysis. [MGI Ref ID J:94759] | ||
Genotyping
Genotyping Information
Genotyping Protocols
Abcg8tm1Elk, Standard PCR
Helpful Links
Genotyping resources and troubleshooting
References
References provided by MGI
Selected Reference(s)
Klett EL; Lu K; Kosters A; Vink E; Lee MH; Altenburg M; Shefer S; Batta AK; Yu H; Chen J; Klein R; Looije N; Oude-Elferink R; Groen AK; Maeda N; Salen G; Patel SB. 2004. A mouse model of sitosterolemia: absence of Abcg8/sterolin-2 results in failure to secrete biliary cholesterol. BMC Med 2(1):5. [PubMed: 15040800] [MGI Ref ID J:94759]
Additional References
Abcg8tm1Elk relatedGroen A; Kunne C; Jongsma G; van den Oever K; Mok KS; Petruzzelli M; Vrins CL; Bull L; Paulusma CC; Oude Elferink RP. 2008. Abcg5/8 independent biliary cholesterol excretion in Atp8b1-deficient mice. Gastroenterology 134(7):2091-100. [PubMed: 18466903] [MGI Ref ID J:136984]
Guan Y; Ackert-Bicknell CL; Kell B; Troyanskaya OG; Hibbs MA. 2010. Functional genomics complements quantitative genetics in identifying disease-gene associations. PLoS Comput Biol 6(11):e1000991. [PubMed: 21085640] [MGI Ref ID J:168495]
Jakulj L; Vissers MN; van Roomen CP; van der Veen JN; Vrins CL; Kunne C; Stellaard F; Kastelein JJ; Groen AK. 2010. Ezetimibe stimulates faecal neutral sterol excretion depending on abcg8 function in mice. FEBS Lett 584(16):3625-8. [PubMed: 20659465] [MGI Ref ID J:163622]
Kosters A; Frijters RJ; Kunne C; Vink E; Schneiders MS; Schaap FG; Nibbering CP; Patel SB; Groen AK. 2005. Diosgenin-induced biliary cholesterol secretion in mice requires Abcg8. Hepatology 41(1):141-50. [PubMed: 15619238] [MGI Ref ID J:105346]
Kosters A; Kunne C; Looije N; Patel SB; Oude Elferink RP; Groen AK. 2006. The mechanism of ABCG5/ABCG8 in biliary cholesterol secretion in mice. J Lipid Res 47(9):1959-66. [PubMed: 16741293] [MGI Ref ID J:114484]
Solca C; Tint GS; Patel SB. 2013. Dietary xenosterols lead to infertility and loss of abdominal adipose tissue in sterolin-deficient mice. J Lipid Res 54(2):397-409. [PubMed: 23180829] [MGI Ref ID J:192806]
van der Velde AE; Vrins CL; van den Oever K; Kunne C; Oude Elferink RP; Kuipers F; Groen AK. 2007. Direct intestinal cholesterol secretion contributes significantly to total fecal neutral sterol excretion in mice. Gastroenterology 133(3):967-75. [PubMed: 17854600] [MGI Ref ID J:128386]
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 & Husbandry When maintaining a live colony, these mice can be bred as homozygotes if maintained on a plant sterol free diet or with drug treatment to block uptake of plant sterols. Homozygous mice on a regular diet exhibit female infertility and rare male infertility.
Pricing and Purchasing
Pricing, Supply Level & Notes, Controls
| Pricing for USA, Canada and Mexico shipping destinations |
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Cryopreserved
Cryopreserved Mice - Ready for Recovery
Animals Provided
Price (US dollars $) Cryorecovery* $1980.00 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 11 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 |
|
Cryopreserved
Cryopreserved Mice - Ready for Recovery
Animals Provided
Price (US dollars $) Cryorecovery* $2574.00 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 11 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).
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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 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's Genotype Promise
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.Ordering Information
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| phone: | 207-288-6470 |
| fax: | 207-288-6655 |
JAX® Mice, Products & Services Conditions of Use
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