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Strain Name:
B6;129S4-G6pc1tm1Jyc/Mmucd
Stock Number:
075773-UCD
Citation ID:
RRID:MMRRC_075773-UCD
Other Names:
G6pc knockout mouse

Strain Information

G6pc1tm1Jyc
Name: glucose-6-phosphatase catalytic subunit 1; targeted mutation 1, Janice Yang Chou
Synonyms: Glc-6-Pase-alpha-, G6Pase-
Type: Allele
Species: Mus musculus (mouse)
Chromosome: 11
Alteration at locus: Knockout
G6pc1
Name: glucose-6-phosphatase catalytic subunit 1
Synonyms: G6Pase, G6pt, Glc-6-Pase, Glc-6-Pase-alpha, G6pc
Type: Gene
Species: Mouse
Chromosome: 11
Alteration at locus: Knockout
NCBI: 14377
HGNC: HGNC:4056
Homologene: 20079
Genetic Alterations
Exon 3 was replaced with a neomycin selection cassette via homologous recombination. Neither transcript nor protein were detected in homozygous mutant mice by a protection assay and Western blot analysis of liver and kidney tissues. Functional ablation was confirmed by the absence of glucose-6-phosphate hydrolysis in liver and kidney microsomes obtained from homozygous mutant mice.
ES Cell Line
J1
Phenotype
G6pc1tm1Jyc (G6Pase-/-) mice manifest the same pathophysiology as human GSD-1a patients, including hypoglycemia, growth retardation, hepatomegaly, kidney enlargement, hyperlipidemia, hyperuricemia, and lactic acidemia.
MeSH Terms
  • Animals
  • Animals, Newborn
  • Base Sequence
  • Biological Transport
  • Blood Glucose/analysis
  • Glucose-6-Phosphatase/genetics
  • Glucose-6-Phosphatase/metabolism
  • Glucose-6-Phosphate
  • Glucosephosphates/genetics
  • Glucosephosphates/metabolism
  • Glycogen Storage Disease Type I/enzymology
  • Glycogen Storage Disease Type I/etiology
  • Glycogen Storage Disease Type I/genetics
  • Kidney/metabolism
  • Kidney/pathology
  • Liver/metabolism
  • Liver/pathology
  • Mice
  • Mice, Knockout
  • Models, Biological
  • Molecular Sequence Data
  • Phenotype
Strain Development
The G6pc1 (G6Pase) gene from the 129Sv/J mouse, spanning approximately 12 kb and containing all five exons was isolated, mapped, and partially sequenced. To disrupt the G6Pase gene, a targeting vector was constructed in which exon 3 and the associated introns of the murine G6Pase gene were replaced with a neomycin (neo) cassette. A herpes simplex virus thymidine kinase (HSV-tk) cassette was also included in the construct for negative selection. The construct was introduced into J1 ES cells (derived from 129S4/SvJae) and the cells were subjected to selection with G418 and gancyclovir. Southern blots of Kpnl and Sful digested genomic DNA were hybridized with 5'-flanking and 3'-flanking probes to identify five targeted clones from 198 doubly resistant clones. Cells from targeted ES cell clones were injected into blastocysts from C57BL/6 mice and transferred into FVB/N foster mothers. Two independent ES clones were used and germline transmission was achieved for each clone. Mice heterozygous for the disrupted G6Pase gene were mated, and homozygous mutant progenies were identified by PCR and Southern blot analysis of tail genomic DNAs.
Suggested Control Mice
age-matched G6pc+/+ and G6pc+/- mice, which have a wild-type phenotype, were used as controls.
MMRRC Genetic QC Summary
The MMRRC Centers have developed a genetic QC pipeline using MiniMUGA array genotyping to provide additional information on strain backgrounds for MMRRC congenic and inbred strains. For more information on when data may be available, or to request genotyping for a strain of interest, please contact mmrrc@ucdavis.edu. Older strains may not have this information.
  • Metabolism
  • Models for Human Disease
Donor
Janice Chou, Ph.D., NICHD/NIH.
Primary Reference

Lei KJ, Chen H, Pan CJ, Ward JM, Mosinger B Jr, Lee EJ, Westphal H, Mansfield BC, Chou JY. Glucose-6-phosphatase dependent substrate transport in the glycogen storage disease type-1a mouse. Nat Genet. 1996 Jun;13(2):203-9. doi: 10.1038/ng0696-203. (Medline PMID: 8640227)

Colony and Husbandry Information

Colony Surveillance Program and Current Health Reports

Mice recovered from a cryo-archive will have health surveillance performed on recipient females. Health reports will be provided prior to shipment. If you require additional health status information, please email mmrrc@ucdavis.edu.
Coat Color
Other
Eye
Black
MMRRC Breeding System
Sib-mating
Generation
N/A
Overall Breeding Performance
Good
Viability and Fertility: Female Male Comments
Homozygotes are viable: Reduced Reduced
Homozygotes are fertile: Undetermined Undetermined
Heterozygotes are fertile: Yes Yes
Age Reproductive Decline: 7 to 9 months 10 to 12 months
Average litter size
7 to 9
Recommended wean age
3 Weeks
Average Pups Weaned
4 to 6

Order Request Information

Limited quantities of breeder mice (recovered litter) are available from a cryoarchive; recovered litter usually available to ship in 3 to 4 months.

Cryopreserved material may be available upon request, please inquire to mmrrc@ucdavis.edu for more information.

A Commercial License Agreement from the Donor is required for for-profit entities to use this strain. For more information, please contact Heather Gunas.

A Commercial License Agreement from the Donor is required for for-profit entities to use this strain. For more information, please contact Heather Gunas

Distribution of this strain requires submission of the MMRRC Conditions of Use (COU). A link to the COU web form will be provided via email after an order has been placed; the form should be completed then or the email forwarded to your institutional official for completion.

Additional charges may apply for any special requests. Shipping costs are in addition to the basic distribution/resuscitation fees. Information on shipping costs and any additional charges will be provided by the supplying MMRRC facility.

Click button to Request this one strain. (Use the MMRRC Catalog Search to request more than one strain.)
MMRRC Item # Description Distribution Fee / Unit (US $)
*Shipping & Handling not included*
Units Notes
075773-UCD-RESUS Litter recovered from cryo-archive $4,044.00 / $7,650.23
Non-Profit / For-Profit
Litter Recovered litter4; additional fees for any special requests.
Cryopreserved material may be available upon request, please inquire to mmrrc@ucdavis.edu for more information.

1 The distribution fee covers the expense of rederiving mice from a live mouse; you will receive the resulting litter. The litter will contain at minimum one mutant carrier; the actual number of animals and the gender and genotype ratios will vary. (Typically, multiple breeder pairs can be established from the recovered litter.) Prior to shipment, the MMRRC will provide information about the animals recovered. If you anticipate or find that you need to request specific genotypes, genders or quantities of mice in excess of what is likely from a resuscitated litter, you may discuss available options and pricing with the supplying MMRRC facility.

2 An aliquot contains a sufficient number of embryos (in one or more vials or straws and based on the transfer success rate of the MMRRC facility) to transfer into one to three recipients. The MMRRC makes no guarantee concerning embryo transfer success experienced in the recipient investigator's laboratory. Neither gender nor genotype ratios are guaranteed.

3 An aliquot is one straw or vial with sufficient sperm to recover at least one litter of mice, as per provided protocols, when performed at the MMRRC facility. The MMRRC makes no guarantee concerning the success of these procedures when performed outside the MMRRC facilities.

4 The distribution fee covers the expense of resuscitating mice from the cryo-archive; you will receive the resulting litter. The litter will contain at minimum one mutant carrier; the actual number of animals and the gender and genotype ratios will vary. (Typically, multiple breeder pairs can be established from the recovered litter.) Prior to shipment, the MMRRC will provide information about the animals recovered. If you anticipate or find that you need to request specific genotypes, genders or quantities of mice in excess of what is likely from a resuscitated litter, you may discuss available options and pricing with the supplying MMRRC facility.

To request material from the MMRRC: Please fill out our on-line request form (accessible from the catalog search results page, or click the Request this Strain button in the fees section). If you have questions or need assistance completing this form, you may call Customer Service at (800) 910-2291 (in USA or Canada) or (530) 757-5710 (international calls). Before you call, please have with you: the MMRRC item number, quantity needed, Bill-to and Ship-to contact information.