Strain Name:

C57BL/6J-Nek8jck/J

Stock Number:

002561

Order this mouse

Availability:

Cryopreserved - Ready for recovery

Other products are available, see Purchasing Information for Cryopreserved Embryos

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 C57BL/6J-jck    (Changed: 15-DEC-04 )
Type Mutant Strain; Spontaneous Mutation;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Specieslaboratory mouse

Appearance
black
Related Genotype: a/a

Description
Mice homozygous for the Nek8jck mutation develop polycystic kidney disease. Histology revealed that the kidneys of some 3 day old pups from heterozygous parents had small isolated cysts lined by cuboidal epithelial cells, and 15 day old pups had cysts lined by flattened epithelia. Disease is progressive but not evident by kidney palpation until at least 4 to 5 weeks of age. Homozygotes generally remain active until shortly before death and usually die between 20 and 25 weeks of age. Homozygous females are fertile but do not consistently care for their litters; homozygous males are fertile but decreased fertility is reported after 15 weeks of age. No histologic abnormalities were found in the liver, spleen, or pancreas. (Atala et al., 1993)

Control Information

  Control
   Wild-type from the colony
   000664 C57BL/6J
 
  Considerations for Choosing Controls

Related Strains

Strains carrying other alleles of Nek8
018199   C57BL/6J-Nek8b2b1449Clo/J
View Strains carrying other alleles of Nek8     (1 strain)

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- Model with phenotypic similarity to human disease where etiologies are distinct. Human genes are associated with this disease. Orthologs of these genes do not appear in the mouse genotype(s).
Polycystic Kidney Disease, Autosomal Recessive; ARPKD
- Potential model based on gene homology relationships. Phenotypic similarity to the human disease has not been tested.
Nephronophthisis 9; NPHP9   (NEK8)
Renal-Hepatic-Pancreatic Dysplasia 2; RHPD2   (NEK8)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Nek8jck/Nek8jck

        involves: C57BL/6J
  • mortality/aging
  • premature death
    • most homozygotes survive to 20 weeks but not longer than 25 weeks   (MGI Ref ID J:11885)
  • renal/urinary system phenotype
  • dilated kidney collecting duct
    • at 7 weeks of age, as shown by MRI imaging   (MGI Ref ID J:79600)
  • enlarged kidney
    • palpable by 7 weeks of age   (MGI Ref ID J:11885)
    • at 7 weeks of age, a 4-fold increase in kidney volume is observed, as assessed by MRI imaging   (MGI Ref ID J:79600)
    • increased kidney weight
      • elevation in ratio of percent kidney weight to body weight at 7 weeks of age   (MGI Ref ID J:213263)
  • kidney cysts
    • E13.5 kidneys grown in vitro for 5 days with 8-Br-cAMP in the culture media display cyst formation, with higher cystic percentage than heterozygous or wild-type kidneys   (MGI Ref ID J:11885)
    • kidney cortex cysts
      • isolated cysts lined with cuboidal epithelial cells are seen by P3   (MGI Ref ID J:11885)
      • by P15, cysts lined with flattened epithelial cells cover about 15% of the cortex region   (MGI Ref ID J:11885)
      • cysts are mostly cortical with some outer medullary involvement; however no cysts are seen in the liver spleen, or pancreas   (MGI Ref ID J:11885)
      • by 7 weeks of age, extensive renal cysts lined by flattened epithelium are found in the kidney cortex   (MGI Ref ID J:79600)
    • kidney medulla cysts
      • by 7 weeks of age, extensive renal cysts lined by flattened epithelium are found in the kidney medulla   (MGI Ref ID J:79600)
    • polycystic kidney
      • by 7 weeks of age, extensive cystic lesions replace normal renal parenchyma   (MGI Ref ID J:79600)
      • cultured kidneys in the presence of 8-bromo-cAMP show 3-fold higher cystogenic potentials than controls and treatment of these cultures with Gant61, a small molecule GLI antagonist, or Sant2, a small molecule SMO antagonist, inhibitors reduces cystogenic potential   (MGI Ref ID J:213263)
  • homeostasis/metabolism phenotype
  • increased circulating creatinine level
    • linear increase in serum creatinine with age from 0.3 mg/dl at 40 days to 1.0 mg/dl at 120 day compared to less than 0.12 mg/dl in controls   (MGI Ref ID J:11885)
  • behavior/neurological phenotype
  • abnormal maternal nurturing
    • females do not consistently care for their pups; however both homozygous males and females are fertile   (MGI Ref ID J:11885)

The following phenotype relates to a compound genotype created using this strain.
Contact JAX® Services jaxservices@jax.org for customized breeding options.

Nek8tm1Bei/Nek8jck

        involves: 129S6/SvEvTac * C57BL/6J * C57BL/6NCr
  • renal/urinary system phenotype
  • abnormal kidney physiology
    • very mild kidney disease   (MGI Ref ID J:199559)
  • enlarged kidney
    • only slightly enlarged   (MGI Ref ID J:199559)
View Research Applications

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

Nek8jck related

Developmental Biology Research
Internal/Organ Defects
      kidney

Internal/Organ Research
Kidney Defects

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Nek8jck
Allele Name juvenile cystic kidney
Allele Type Spontaneous
Common Name(s) Nek8-; jck;
Strain of OriginC57BL/6J Tg 147-9a
Gene Symbol and Name Nek8, NIMA (never in mitosis gene a)-related expressed kinase 8
Chromosome 11
Gene Common Name(s) 4632401F23Rik; JCK; Mutant line 1449; NEK12A; NPHP9; RHPD2; RIKEN cDNA 4632401F23 gene; b2b1449Clo; jck; juvenile cystic kidney;
Molecular Note The mutation in jck mice has been identified as a nucleotide substitution (G1348T), resulting in a nonconservative amino acid substitution of valine for glycine at amino acid 488 in the C-terminal domain of the protein. [MGI Ref ID J:79896]

Genotyping

Genotyping Information

Genotyping Protocols

Nek8jck, Pyrosequencing
Nek8jck, Restriction Enzyme Digest


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Atala A; Freeman MR; Mandell J; Beier DR. 1993. Juvenile cystic kidneys (jck): a new mouse mutation which causes polycystic kidneys. Kidney Int 43(5):1081-5. [PubMed: 8510385]  [MGI Ref ID J:11885]

Additional References

Liu S; Lu W; Obara T; Kuida S; Lehoczky J; Dewar K; Drummond IA; Beier DR. 2002. A defect in a novel Nek-family kinase causes cystic kidney disease in the mouse and in zebrafish. Development 129(24):5839-46. [PubMed: 12421721]  [MGI Ref ID J:79896]

Surendran K; McCaul SP; Simon TC. 2002. A role for Wnt-4 in renal fibrosis. Am J Physiol Renal Physiol 282(3):F431-41. [PubMed: 11832423]  [MGI Ref ID J:75544]

Nek8jck related

Choi HJ; Lin JR; Vannier JB; Slaats GG; Kile AC; Paulsen RD; Manning DK; Beier DR; Giles RH; Boulton SJ; Cimprich KA. 2013. NEK8 links the ATR-regulated replication stress response and S phase CDK activity to renal ciliopathies. Mol Cell 51(4):423-39. [PubMed: 23973373]  [MGI Ref ID J:203943]

Guay-Woodford LM. 2003. Murine models of polycystic kidney disease: molecular and therapeutic insights. Am J Physiol Renal Physiol 285(6):F1034-49. [PubMed: 14600027]  [MGI Ref ID J:87130]

Hellman NE; Liu Y; Merkel E; Austin C; Le Corre S; Beier DR; Sun Z; Sharma N; Yoder BK; Drummond IA. 2010. The zebrafish foxj1a transcription factor regulates cilia function in response to injury and epithelial stretch. Proc Natl Acad Sci U S A 107(43):18499-504. [PubMed: 20937855]  [MGI Ref ID J:165518]

Hoff S; Halbritter J; Epting D; Frank V; Nguyen TM; van Reeuwijk J; Boehlke C; Schell C; Yasunaga T; Helmstadter M; Mergen M; Filhol E; Boldt K; Horn N; Ueffing M; Otto EA; Eisenberger T; Elting MW; van Wijk JA; Bockenhauer D; Sebire NJ; Rittig S; VybergM; Ring T; Pohl M; Pape L; Neuhaus TJ; Elshakhs NA; Koon SJ; Harris PC; Grahammer F; Huber TB; Kuehn EW; Kramer-Zucker A; Bolz HJ; Roepman R; Saunier S; Walz G; Hildebrandt F; Bergmann C; Lienkamp SS. 2013. ANKS6 is a central component of a nephronophthisis module linking NEK8 to INVS and NPHP3. Nat Genet 45(8):951-6. [PubMed: 23793029]  [MGI Ref ID J:205163]

Iakoubova O; Dushkin H; Pacella L; Beier DR. 1999. Genetic analysis of modifying loci on mouse chromosome 1 that affect disease severity in a model of recessive PKD. Physiol Genomics 1(2):101-5. [PubMed: 11015567]  [MGI Ref ID J:59425]

Iakoubova OA; Dushkin H; Beier DR. 1997. Genetic analysis of a quantitative trait in a mouse model of polycystic kidney disease. Am J Respir Crit Care Med 156(4 Pt 2):S72-7. [PubMed: 9351583]  [MGI Ref ID J:43909]

Iakoubova OA; Dushkin H; Beier DR. 1995. Localization of a murine recessive polycystic kidney disease mutation and modifying loci that affect disease severity. Genomics 26(1):107-14. [PubMed: 7782068]  [MGI Ref ID J:23893]

Kuida S; Beier DR. 2000. Genetic localization of interacting modifiers affecting severity in a murine model of polycystic kidney disease. Genome Res 10(1):49-54. [PubMed: 10645949]  [MGI Ref ID J:60140]

Liu S; Lu W; Obara T; Kuida S; Lehoczky J; Dewar K; Drummond IA; Beier DR. 2002. A defect in a novel Nek-family kinase causes cystic kidney disease in the mouse and in zebrafish. Development 129(24):5839-46. [PubMed: 12421721]  [MGI Ref ID J:79896]

Manning DK; Sergeev M; van Heesbeen RG; Wong MD; Oh JH; Liu Y; Henkelman RM; Drummond I; Shah JV; Beier DR. 2013. Loss of the ciliary kinase Nek8 causes left-right asymmetry defects. J Am Soc Nephrol 24(1):100-12. [PubMed: 23274954]  [MGI Ref ID J:199559]

Natoli TA; Gareski TC; Dackowski WR; Smith L; Bukanov NO; Russo RJ; Husson H; Matthews D; Piepenhagen P; Ibraghimov-Beskrovnaya O. 2008. Pkd1 and Nek8 mutations affect cell-cell adhesion and cilia in cysts formed in kidney organ cultures. Am J Physiol Renal Physiol 294(1):F73-83. [PubMed: 17928412]  [MGI Ref ID J:130086]

Natoli TA; Smith LA; Rogers KA; Wang B; Komarnitsky S; Budman Y; Belenky A; Bukanov NO; Dackowski WR; Husson H; Russo RJ; Shayman JA; Ledbetter SR; Leonard JP; Ibraghimov-Beskrovnaya O. 2010. Inhibition of glucosylceramide accumulation results in effective blockade of polycystic kidney disease in mouse models. Nat Med 16(7):788-92. [PubMed: 20562878]  [MGI Ref ID J:162080]

Pollard R; Yunis R; Kultz D; Martin P; Griffey S; Ferrara K. 2006. Ultrasound detection and characterization of polycystic kidney disease in a mouse model. Comp Med 56(3):215-21. [PubMed: 16774131]  [MGI Ref ID J:144893]

Smith LA; Bukanov NO; Husson H; Russo RJ; Barry TC; Taylor AL; Beier DR; Ibraghimov-Beskrovnaya O. 2006. Development of polycystic kidney disease in juvenile cystic kidney mice: insights into pathogenesis, ciliary abnormalities, and common features with human disease. J Am Soc Nephrol 17(10):2821-31. [PubMed: 16928806]  [MGI Ref ID J:135934]

Sohara E; Luo Y; Zhang J; Manning DK; Beier DR; Zhou J. 2008. Nek8 regulates the expression and localization of polycystin-1 and polycystin-2. J Am Soc Nephrol 19(3):469-76. [PubMed: 18235101]  [MGI Ref ID J:149929]

Sun Y; Zhou J; Stayner C; Munasinghe J; Shen X; Beier DR; Albert MS. 2002. Magnetic resonance imaging assessment of a murine model of recessive polycystic kidney disease. Comp Med 52(5):433-8. [PubMed: 12405636]  [MGI Ref ID J:79600]

Tran PV; Talbott GC; Turbe-Doan A; Jacobs DT; Schonfeld MP; Silva LM; Chatterjee A; Prysak M; Allard BA; Beier DR. 2014. Downregulating Hedgehog Signaling Reduces Renal Cystogenic Potential of Mouse Models. J Am Soc Nephrol :. [PubMed: 24700869]  [MGI Ref ID J:213263]

Trapp ML; Galtseva A; Manning DK; Beier DR; Rosenblum ND; Quarmby LM. 2008. Defects in ciliary localization of Nek8 is associated with cystogenesis. Pediatr Nephrol 23(3):377-87. [PubMed: 18189147]  [MGI Ref ID J:134562]

Viau A; El Karoui K; Laouari D; Burtin M; Nguyen C; Mori K; Pillebout E; Berger T; Mak TW; Knebelmann B; Friedlander G; Barasch J; Terzi F. 2010. Lipocalin 2 is essential for chronic kidney disease progression in mice and humans. J Clin Invest 120(11):4065-76. [PubMed: 20921623]  [MGI Ref ID J:166941]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, G200.

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.

General Supply Notes

  • View the complete collection of spontaneous mutants in the Mouse Mutant Resource.

Control Information

  Control
   Wild-type from the colony
   000664 C57BL/6J
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

Payment Terms and Conditions

Terms are granted by individual review and stated on the customer invoice(s) and account statement. These transactions are payable in U.S. currency within the granted terms. Payment for services, products, shipping containers, and shipping costs that are rendered are expected within the payment terms indicated on the invoice or stated by contract. Invoices and account balances in arrears of stated terms may result in The Jackson Laboratory pursuing collection activities including but not limited to outside agencies and court filings.


See Terms of Use tab for General Terms and Conditions


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
JAX® Mice
Surgical and Preconditioning Services
JAX® Services
Customer Services and Support
Tel: 1-800-422-6423 or 1-207-288-5845
Fax: 1-207-288-6150
Technical Support Email Form

Terms of Use

Terms of Use


General Terms and Conditions


Contact information

General inquiries regarding Terms of Use

Contracts Administration

phone:207-288-6470

JAX® Mice, Products & Services Conditions of Use

"MICE" means mouse strains, their progeny derived by inbreeding or crossbreeding, unmodified derivatives from mouse strains or their progeny supplied by The Jackson Laboratory ("JACKSON"). "PRODUCTS" means biological materials supplied by JACKSON, and their derivatives. "RECIPIENT" means each recipient of MICE, PRODUCTS, or services provided by JACKSON including each institution, its employees and other researchers under its control. MICE or PRODUCTS shall not be: (i) used for any purpose other than the internal research, (ii) sold or otherwise provided to any third party for any use, or (iii) provided to any agent or other third party to provide breeding or other services. Acceptance of MICE or PRODUCTS from JACKSON shall be deemed as agreement by RECIPIENT to these conditions, and departure from these conditions requires JACKSON's prior written authorization.

No Warranty

MICE, PRODUCTS AND SERVICES ARE PROVIDED “AS IS”. JACKSON EXTENDS NO WARRANTIES OF ANY KIND, EITHER EXPRESS, IMPLIED, OR STATUTORY, WITH RESPECT TO MICE, PRODUCTS OR SERVICES, INCLUDING ANY IMPLIED WARRANTY OF MERCHANTABILITY OR FITNESS FOR A PARTICULAR PURPOSE, OR ANY WARRANTY OF NON-INFRINGEMENT OF ANY PATENT, TRADEMARK, OR OTHER INTELLECTUAL PROPERTY RIGHTS.

In case of dissatisfaction for a valid reason and claimed in writing by a purchaser within ninety (90) days of receipt of mice, products or services, JACKSON will, at its option, provide credit or replacement for the mice or product received or the services provided.

No Liability

In no event shall JACKSON, its trustees, directors, officers, employees, and affiliates be liable for any causes of action or damages, including any direct, indirect, special, or consequential damages, arising out of the provision of MICE, PRODUCTS or services, including economic damage or injury to property and lost profits, and including any damage arising from acts or negligence on the part of JACKSON, its agents or employees. Unless prohibited by law, in purchasing or receiving MICE, PRODUCTS or services from JACKSON, purchaser or recipient, or any party claiming by or through them, expressly releases and discharges JACKSON from all such causes of action or damages, and further agrees to defend and indemnify JACKSON from any costs or damages arising out of any third party claims.

MICE and PRODUCTS are to be used in a safe manner and in accordance with all applicable governmental rules and regulations.

The foregoing represents the General Terms and Conditions applicable to JACKSON’s MICE, PRODUCTS or services. In addition, special terms and conditions of sale of certain MICE, PRODUCTS or services may be set forth separately in JACKSON web pages, catalogs, price lists, contracts, and/or other documents, and these special terms and conditions shall also govern the sale of these MICE, PRODUCTS and services by JACKSON, and by its licensees and distributors.

Acceptance of delivery of MICE, PRODUCTS or services shall be deemed agreement to these terms and conditions. No purchase order or other document transmitted by purchaser or recipient that may modify the terms and conditions hereof, shall be in any way binding on JACKSON, and instead the terms and conditions set forth herein, including any special terms and conditions set forth separately, shall govern the sale of MICE, PRODUCTS or services by JACKSON.


(6.8)