Strain Name:

B6.129S4-Pkd1tm2Ggg/J

Stock Number:

010671

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These floxed Pkd1 (polycystic kidney disease 1 homolog) mutant mice may be useful in generating conditional mutations for studies of polycystic kidney disease.

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.
Visit our online Nomenclature tutorial.
Additional information on Congenic nomenclature.
Specieslaboratory mouse
GenerationN10F?pN1
Generation Definitions
 
Donating Investigator Gregory Germino,   Johns Hopkins University School of Medic

Description
These mice possess loxP sites on either side of exons 2 through 4 of the targeted gene. Mice that are homozygous for this allele are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. When these mutant mice are bred to mice that express Cre recombinase, resulting offspring will have exons 2 through 4 deleted in the cre-expressing tissue(s).

For example, when crossed to a strain with widespread Cre recombinase expression (see Stock No. 003553), this mutant mouse strain may be useful in studies of polycystic kidneys.

Development
A targeting vector containing a FRT-site flanked neo cassette and a loxP site was utilized in the construction of this mutant. This selection cassette was inserted upstream of exon 2 of the targeted gene, and another loxP site was inserted upstream of exon 4. This construct was electroporated into 129S4/SvJae derived J1 embryonic stem (ES) cells. Correctly targeted ES cells were injected into C57BL/6 blastocysts. The mice were then crossed to a FLP recombinase expressing strain, on a congenic C57BL/6J background, to remove the selection cassette. Mice that had successfully undergone FLP recombination and no longer retained the cassette but did retain the loxP-flanked exons 2-4 were then backcrossed to C57BL/6 for 12 generations (see SNP note below). Heterozygotes were crossed to generate homozygotes. Upon arrival at The Jackson Laboratory the mice were crossed to C57BL/6J at least once to establish the colony.

A 32 SNP (single nucleotide polymorphism) panel analysis, with 27 markers covering all 19 chromosomes and the X chromosome, as well as 5 markers that distinguish between the C57BL/6J and C57BL/6N substrains, was performed on the rederived living colony at The Jackson Laboratory Repository. While the 27 markers throughout the genome suggested a C57BL/6 genetic background, at least 1 of 5 markers that determine C57BL/6J from C57BL/6N were found to be segregating. These data suggest the mice sent to The Jackson Laboratory Repository were on a mixed C57BL/6J ; C57BL/6N genetic background.

Control Information

  Control
   000664 C57BL/6J
 
  Considerations for Choosing Controls

Related Strains

Strains carrying other alleles of Pkd1
018189   C57BL/6J-Pkd1b2b1585Clo/J
View Strains carrying other alleles of Pkd1     (1 strain)

Additional Web Information

Introduction to Cre-lox technology

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- Model with phenotypic similarity to human disease where etiologies involve orthologs. Human genes are associated with this disease. Orthologs of those genes appear in the mouse genotype(s).
Polycystic Kidney Disease 1; PKD1
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

The following phenotype relates to a compound genotype created using this strain.
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Pkd1tm2Ggg/Pkd1tm2Ggg Tg(MMTV-cre)4Mam/0

        involves: 129S4/SvJae * FVB   (conditional)
  • renal/urinary system phenotype
  • kidney cysts
    • 60% exhibit an occasional cyst on the surface of the kidney at 10 weeks of age and 100% have kidney cysts at 20 weeks of age   (MGI Ref ID J:103719)
  • liver/biliary system phenotype
  • liver cysts
    • 20% exhibit an occasional cyst on the surface of the liver at 10 weeks of age and 100% have multiple hepatic cysts at 20 weeks of age   (MGI Ref ID J:103719)
View Research Applications

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

Research Tools
Cre-lox System
      loxP-flanked Sequences

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Pkd1tm2Ggg
Allele Name targeted mutation 2, Gregory G Germino
Allele Type Targeted (Conditional ready (e.g. floxed), No functional change)
Common Name(s) Pkd1cond; Pkd1flox;
Mutation Made By Gregory Germino,   Johns Hopkins University School of Medic
Strain of Origin129S4/SvJae
Gene Symbol and Name Pkd1, polycystic kidney disease 1 homolog
Chromosome 17
Gene Common Name(s) PBP; PC-1; PC1; TRPP1; polycystin-1;
Molecular Note A neomycin cassette flanked by FRT sites and a single loxP site at the 3' end was inserted into intron 1. Another loxP site was inserted into intron 4 in the same orientation such that exons 2-4 could be removed upon expression of cre recombinase. [MGI Ref ID J:103719]

Genotyping

Genotyping Information

Genotyping Protocols

Pkd1tm2Ggg, Standard PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Piontek KB; Huso DL; Grinberg A; Liu L; Bedja D; Zhao H; Gabrielson K; Qian F; Mei C; Westphal H; Germino GG. 2004. A functional floxed allele of Pkd1 that can be conditionally inactivated in vivo. J Am Soc Nephrol 15(12):3035-43. [PubMed: 15579506]  [MGI Ref ID J:103719]

Additional References

Pkd1tm2Ggg related

Foy RL; Chitalia VC; Panchenko MV; Zeng L; Lopez D; Lee JW; Rana SV; Boletta A; Qian F; Tsiokas L; Piontek KB; Germino GG; Zhou MI; Cohen HT. 2012. Polycystin-1 regulates the stability and ubiquitination of transcription factor Jade-1. Hum Mol Genet 21(26):5456-71. [PubMed: 23001567]  [MGI Ref ID J:191148]

Garcia-Gonzalez MA; Outeda P; Zhou Q; Zhou F; Menezes LF; Qian F; Huso DL; Germino GG; Piontek KB; Watnick T. 2010. Pkd1 and Pkd2 are required for normal placental development. PLoS One 5(9):. [PubMed: 20862291]  [MGI Ref ID J:165114]

Menezes LF; Zhou F; Patterson AD; Piontek KB; Krausz KW; Gonzalez FJ; Germino GG. 2012. Network analysis of a Pkd1-mouse model of autosomal dominant polycystic kidney disease identifies HNF4alpha as a disease modifier. PLoS Genet 8(11):e1003053. [PubMed: 23209428]  [MGI Ref ID J:194672]

Nie X; Arend LJ. 2013. Pkd1 is required for male reproductive tract development. Mech Dev 130(11-12):567-76. [PubMed: 23933588]  [MGI Ref ID J:204460]

Olsan EE; Mukherjee S; Wulkersdorfer B; Shillingford JM; Giovannone AJ; Todorov G; Song X; Pei Y; Weimbs T. 2011. Signal transducer and activator of transcription-6 (STAT6) inhibition suppresses renal cyst growth in polycystic kidney disease. Proc Natl Acad Sci U S A 108(44):18067-72. [PubMed: 22025716]  [MGI Ref ID J:180251]

Piontek K; Menezes LF; Garcia-Gonzalez MA; Huso DL; Germino GG. 2007. A critical developmental switch defines the kinetics of kidney cyst formation after loss of Pkd1. Nat Med 13(12):1490-5. [PubMed: 17965720]  [MGI Ref ID J:141463]

Qiu N; Cao L; David V; Quarles LD; Xiao Z. 2010. Kif3a deficiency reverses the skeletal abnormalities in Pkd1 deficient mice by restoring the balance between osteogenesis and adipogenesis. PLoS One 5(12):e15240. [PubMed: 21151991]  [MGI Ref ID J:167728]

Qiu N; Xiao Z; Cao L; David V; Quarles LD. 2012. Conditional mesenchymal disruption of pkd1 results in osteopenia and polycystic kidney disease. PLoS One 7(9):e46038. [PubMed: 23029375]  [MGI Ref ID J:191967]

Raphael KL; Strait KA; Stricklett PK; Miller RL; Nelson RD; Piontek KB; Germino GG; Kohan DE. 2009. Inactivation of Pkd1 in principal cells causes a more severe cystic kidney disease than in intercalated cells. Kidney Int 75(6):626-33. [PubMed: 19145237]  [MGI Ref ID J:162703]

Seeger-Nukpezah T; Proia DA; Egleston BL; Nikonova AS; Kent T; Cai KQ; Hensley HH; Ying W; Chimmanamada D; Serebriiskii IG; Golemis EA. 2013. Inhibiting the HSP90 chaperone slows cyst growth in a mouse model of autosomal dominant polycystic kidney disease. Proc Natl Acad Sci U S A 110(31):12786-91. [PubMed: 23858461]  [MGI Ref ID J:198775]

Shillingford JM; Murcia NS; Larson CH; Low SH; Hedgepeth R; Brown N; Flask CA; Novick AC; Goldfarb DA; Kramer-Zucker A; Walz G; Piontek KB; Germino GG; Weimbs T. 2006. The mTOR pathway is regulated by polycystin-1, and its inhibition reverses renal cystogenesis in polycystic kidney disease. Proc Natl Acad Sci U S A 103(14):5466-71. [PubMed: 16567633]  [MGI Ref ID J:108293]

Shillingford JM; Piontek KB; Germino GG; Weimbs T. 2010. Rapamycin ameliorates PKD resulting from conditional inactivation of Pkd1. J Am Soc Nephrol 21(3):489-97. [PubMed: 20075061]  [MGI Ref ID J:185855]

Steigelman KA; Lelli A; Wu X; Gao J; Lin S; Piontek K; Wodarczyk C; Boletta A; Kim H; Qian F; Germino G; Geleoc GS; Holt JR; Zuo J. 2011. Polycystin-1 is required for stereocilia structure but not for mechanotransduction in inner ear hair cells. J Neurosci 31(34):12241-50. [PubMed: 21865467]  [MGI Ref ID J:176236]

Talbot JJ; Shillingford JM; Vasanth S; Doerr N; Mukherjee S; Kinter MT; Watnick T; Weimbs T. 2011. Polycystin-1 regulates STAT activity by a dual mechanism. Proc Natl Acad Sci U S A 108(19):7985-90. [PubMed: 21518865]  [MGI Ref ID J:172196]

Xiao Z; Dallas M; Qiu N; Nicolella D; Cao L; Johnson M; Bonewald L; Quarles LD. 2011. Conditional deletion of Pkd1 in osteocytes disrupts skeletal mechanosensing in mice. FASEB J 25(7):2418-32. [PubMed: 21454365]  [MGI Ref ID J:174324]

Xiao Z; Zhang S; Cao L; Qiu N; David V; Quarles LD. 2010. Conditional disruption of Pkd1 in osteoblasts results in osteopenia due to direct impairment of bone formation. J Biol Chem 285(2):1177-87. [PubMed: 19887454]  [MGI Ref ID J:159961]

Zhou X; Fan LX; Li K; Ramchandran R; Calvet JP; Li X. 2014. SIRT2 regulates ciliogenesis and contributes to abnormal centrosome amplification caused by loss of polycystin-1. Hum Mol Genet 23(6):1644-55. [PubMed: 24203696]  [MGI Ref ID J:206980]

Zhou X; Fan LX; Sweeney WE Jr; Denu JM; Avner ED; Li X. 2013. Sirtuin 1 inhibition delays cyst formation in autosomal-dominant polycystic kidney disease. J Clin Invest 123(7):3084-98. [PubMed: 23778143]  [MGI Ref ID J:201423]

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 can be bred as homozygotes.

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* $2140.00
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 willfulfill 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* $2782.00
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 willfulfill 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
   000664 C57BL/6J
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

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