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. Species laboratory mouse Generation N12p (21-AUG-05) Generation Definitions

Appearance
black
Related Genotype: a/a

Description
Mice homozygous for the Nek8^jck 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)

View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI

      assigned by genotype

Nek8^jck/Nek8^jck

        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)
• 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)
• 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)

View Research Applications

Research Applications

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

Nek8^jck 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 Origin		C57BL/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; 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

Nek8^jck, Pyrosequencing
Nek8^jck, 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]

Nek8^jck related

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]

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]

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]

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

General Supply Notes

• View the complete collection of spontaneous mutants in the Mouse Mutant Resource.
• Genomic DNA is available for this strain from the Mouse DNA Resource.

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 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.

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