Strain Name:

B6.RBF(C3Fe)-Nek1kat/J

Stock Number:

001271

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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.RBF-Nek1kat/J    (Changed: 11-FEB-09 )
Type Congenic; Mutant Strain;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Additional information on Congenic nomenclature.
Specieslaboratory mouse and M. m. domesticus
GenerationN6
Generation Definitions

Description
The phenotype associated with the Nek1kat-2J allele is more severe than that of the Nek1kat allele. Mice homozygous for Nek1kat-2J all die before one year of age, a third of those that survive weaning die suddenly before 100 days of age, and the reported median survival age is 211 days. Mice homozygous for Nek1kat also have a shortened life expectancy with high pre-weaning mortality and a reported median survival of 286 days, but 22% were found to survive beyond 1 year. Both mutants are runted and have blunted noses with olfactory bulbs that are approximately half the normal size and lack most of the glomerular and external granular layers. In the brain there is dilation of the lateral and third ventricles, cerebral aqueduct and fourth ventricle along with large, fluid-filled cysts in the choroid plexus. Hydrocephalus occurs. Normochromic normocytic anemia is found and is more pronounced in Nek1Kat-2J homozygotes, often detectable before weaning and progressing with age. By 7 to 8 months of age uremia with abnormally shaped erythrocytes can also be detected in the peripheral blood. The hematocrit levels of Nek1kat homozygotes is lower than normal in the young, but a significant drop occurs after approximately 200 days of age then continues to decrease. Abdominal distention occurs as a result of extensive renal enlargement. At 5 days of age microscopic examination fails to detect any morphologic change in the kidneys of either mutant, but fluid-filled cysts and dilated proximal tubules and Bowman spaces are found as early as 1 month of age in Nek1kat-2J homozygotes. The bilateral renal cystic disease progresses involving all levels of the nephron by 3 months of age, and after 6 months of age this progression becomes comparatively more rapid in the females. Disease progression includes growth of cysts and an increase in the number of cysts. Nek1kat homozygotes have similar renal cystic disease but the onset is slower relative to that in Nek1kat-2J homozygotes. (For morphologic details see Vogler et al., 1999.) Testicular hypoplasia with decreased spermatogenesis has been described for Nek1kat-2J homozygotes, and male sterility is a characteristic of both Nek1 mutants. No histological abnormalities have been described in the ovaries and homozygous females can reproduce but litters are less frequent than from wildtype or heterozygous females. Focal portal bile duct proliferation and dilation have been found in the older Nek1Kat-2J homozygotes. (Janaswami et al., 1997; Vogler et al., 1999.)

Development
The kat allele arose spontaneously on the RBF/Dn background, then at F66, at The Jackson Laboratory in 1986. It was backcrossed to C3HeB/FeJ three times then subsequently backcrossed to C57BL/6J and in heterozygous N5 and N6 males were bred to C57BL/6J females to generate embryos for cryopreservation.

Related Strains

Strains carrying other alleles of Nek1
002854   C57BL/6J-Nek1kat-2J/J
View Strains carrying other alleles of Nek1     (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.
Short-Rib Thoracic Dysplasia 6 with or without Polydactyly; SRTD6   (NEK1)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Nek1kat/Nek1kat

        B6.RBF(C3Fe)-Nek1kat/J
  • mortality/aging
  • partial postnatal lethality
    • by generation N5 on the C57BL/6J background half of the homozygotes die before weaning   (MGI Ref ID J:37799)
  • growth/size/body phenotype
  • decreased body size
    • by generation N5 to C57BL/6J there is increased severity of the phenotype including severe dwarfing   (MGI Ref ID J:37799)
    • proportional dwarf   (MGI Ref ID J:37799)
  • craniofacial phenotype
  • domed cranium   (MGI Ref ID J:37799)
  • skeleton phenotype
  • domed cranium   (MGI Ref ID J:37799)

The following phenotype information is associated with a similar, but not exact match to this JAX® Mice strain.

Nek1kat/Nek1kat

        RBF/Dn
  • mortality/aging
  • postnatal lethality
    • on the RBF/Dn background, but not the C3HeB/FeJLe x RBF/Dn mixed background, there is a biphasic death pattern wherein approximately half the homozygotes die before weaning and the remainder die by 1 year of age   (MGI Ref ID J:37799)
  • premature death   (MGI Ref ID J:37799)
  • craniofacial phenotype
  • domed cranium   (MGI Ref ID J:37799)
  • growth/size/body phenotype
  • decreased body size
    • all homozygotes are runted with shortened bodies   (MGI Ref ID J:37799)
    • decreased body length   (MGI Ref ID J:37799)
  • renal/urinary system phenotype
  • kidney cysts   (MGI Ref ID J:37799)
  • skeleton phenotype
  • domed cranium   (MGI Ref ID J:37799)
  • integument phenotype
  • sparse hair   (MGI Ref ID J:37799)

Nek1kat/Nek1kat

        involves: C3HeB/FeJLe * RBF/Dn
  • mortality/aging
  • premature death
    • on this mixed genetic background homozygotes survive to adulthood, but still have a shortened lifespan   (MGI Ref ID J:37799)
  • reproductive system phenotype
  • male infertility   (MGI Ref ID J:37799)
  • reduced female fertility
    • homozygous females on this mixed background only breed occasionally   (MGI Ref ID J:37799)
  • growth/size/body phenotype
  • decreased body size   (MGI Ref ID J:37799)
    • decreased body length   (MGI Ref ID J:37799)

Nek1kat/Nek1kat

        involves: C3HeB/FeJLe * C57BL/6J * RBF/Dn
  • mortality/aging
  • premature death
    • 22% survive beyond 1 year   (MGI Ref ID J:59839)
  • renal/urinary system phenotype
  • kidney cysts   (MGI Ref ID J:59839)
    • bilateral polycystic kidney disease with a progressive increase in the number and size of cysts in the kidney cortex, with slower progression than in kat-2J homozygotes   (MGI Ref ID J:37799)
    • cysts seem to be dilated proximal tubules and Bowman spaces   (MGI Ref ID J:37799)
    • kidney cortex cysts   (MGI Ref ID J:37799)
    • polycystic kidney   (MGI Ref ID J:37799)
  • nervous system phenotype
  • abnormal cerebral aqueduct morphology   (MGI Ref ID J:37799)
  • abnormal choroid plexus morphology
    • at 7 months of age the choroid plexus has multiple large cysts present in the interstitium   (MGI Ref ID J:37799)
  • abnormal olfactory bulb morphology
    • most of the olfactory glomerular and external granular layers are absent, and in places are replaced by lacy vacuolated tissue   (MGI Ref ID J:37799)
    • small olfactory bulb
      • olfactory lobes are approximately half normal size   (MGI Ref ID J:37799)
  • dilated fourth ventricle
    • at 7 months of age the lateral, third, and fourth ventricles and cerebral aqueduct are dilated   (MGI Ref ID J:37799)
  • dilated lateral ventricles
    • at 7 months of age the lateral, third, and fourth ventricles and cerebral aqueduct are dilated   (MGI Ref ID J:37799)
  • dilated third ventricle
    • at 7 months of age the lateral, third, and fourth ventricles and cerebral aqueduct are dilated   (MGI Ref ID J:37799)
  • obstructive hydrocephaly   (MGI Ref ID J:37799)
  • growth/size/body phenotype
  • decreased body size   (MGI Ref ID J:59839)
    • decreased body length   (MGI Ref ID J:59839)
  • craniofacial phenotype
  • abnormal craniofacial morphology
    • foreshortened face, particularly the snout   (MGI Ref ID J:59839)
    • short snout   (MGI Ref ID J:59839)
    • shortened head   (MGI Ref ID J:59839)
  • hematopoietic system phenotype
  • abnormal erythrocyte osmotic lysis   (MGI Ref ID J:59839)
  • anemia
    • hematocrit is slightly reduced in many young homozygotes and falls progressively after 200 days of age   (MGI Ref ID J:59839)
View Research Applications

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

Nek1kat related

Developmental Biology Research
Craniofacial and Palate Defects
Growth Defects
Internal/Organ Defects
      brain
      gonads
      kidney
Neurodevelopmental Defects
Postnatal Lethality

Hematological Research
Anemia, Iron Deficiency and Transport Defects

Internal/Organ Research
Kidney Defects

Neurobiology Research
Neurodevelopmental Defects

Reproductive Biology Research
Developmental Defects Affecting Gonads
Fertility Defects

Sensorineural Research
Olfactory Defects

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Nek1kat
Allele Name kidney, anemia and testis
Allele Type Spontaneous
Common Name(s) kat;
Strain of OriginRBF/Dn
Gene Symbol and Name Nek1, NIMA (never in mitosis gene a)-related expressed kinase 1
Chromosome 8
Gene Common Name(s) D8Ertd790e; DNA segment, Chr 8, ERATO Doi 790, expressed; NY-REN-55; SRPS2; SRPS2A; SRTD6; kat; kidney, anemia and testis;
General Note Homozygous mutant mice have a latent onset, slowly progressing form of polycystic kidney disease (PKD) with renal pathology similar to the human autosomal-dominant PKD OMIM 173900, OMIM 600666). In addition, mutant mice show pleiotropic effects that include facial dysmorphism, dwarfing, male sterility, anemia, and cystic choroid plexus (J:59363).
Molecular Note Nucleotides 791-2105 are deleted. [MGI Ref ID J:59363]

Genotyping

Genotyping Information


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Additional References

Arama E; Yanai A; Kilfin G; Bernstein A; Motro B. 1998. Murine NIMA-related kinases are expressed in patterns suggesting distinct functions in gametogenesis and a role in the nervous system. Oncogene 16(14):1813-23. [PubMed: 9583679]  [MGI Ref ID J:47160]

Janaswami PM; Birkenmeier EH; Cook SA; Rowe LB; Bronson RT; Davisson MT. 1997. Identification and genetic mapping of a new polycystic kidney disease on mouse chromosome 8. Genomics 40(1):101-7. [PubMed: 9070925]  [MGI Ref ID J:37799]

Letwin K; Mizzen L; Motro B; Ben-David Y; Bernstein A; Pawson T. 1992. A mammalian dual specificity protein kinase, Nek1, is related to the NIMA cell cycle regulator and highly expressed in meiotic germ cells. EMBO J 11(10):3521-31. [PubMed: 1382974]  [MGI Ref ID J:2376]

Tanaka K; Parvinen M; Nigg EA. 1997. The in vivo expression pattern of mouse Nek2, a NIMA-related kinase, indicates a role in both mitosis and meiosis. Exp Cell Res 237(2):264-74. [PubMed: 9434622]  [MGI Ref ID J:45011]

Upadhya P; Birkenmeier EH; Birkenmeier CS; Barker JE. 2000. Mutations in a NIMA-related kinase gene, Nek1, cause pleiotropic effects including a progressive polycystic kidney disease in mice. Proc Natl Acad Sci U S A 97(1):217-21. [PubMed: 10618398]  [MGI Ref ID J:59363]

Nek1kat 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]

Janaswami PM; Birkenmeier EH; Cook SA; Rowe LB; Bronson RT; Davisson MT. 1997. Identification and genetic mapping of a new polycystic kidney disease on mouse chromosome 8. Genomics 40(1):101-7. [PubMed: 9070925]  [MGI Ref ID J:37799]

Upadhya P; Birkenmeier EH; Birkenmeier CS; Barker JE. 2000. Mutations in a NIMA-related kinase gene, Nek1, cause pleiotropic effects including a progressive polycystic kidney disease in mice. Proc Natl Acad Sci U S A 97(1):217-21. [PubMed: 10618398]  [MGI Ref ID J:59363]

Vogler C; Homan S; Pung A; Thorpe C; Barker J; Birkenmeier EH; Upadhya P. 1999. Clinical and pathologic findings in two new allelic murine models of polycystic kidney disease. J Am Soc Nephrol 10(12):2534-9. [PubMed: 10589692]  [MGI Ref ID J:59839]

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* $3175.00
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 of Strains Needing Progeny Testing
    At least two untested males and two untested females (two pairs) will be recovered (eight or more mice is typical). The total number of animals provided, their gender and genotype will vary. Untested animals typically are available to ship between 13 and 16 weeks from the date of your order. If the first recovery attempt is unsuccessful, a second recovery will be done, extending the overall recovery time to approximately 25 weeks. Progeny testing is required to identify the genotype of mice of this strain, as a genotyping assay is not available. This type of testing involves breeding the recovered animals and assessing the phenotype of the offspring in order to identify animals carrying the mutation of interest. We can perform the progeny testing for you as a service or we can ship all recovered animals to you for progeny testing at your facility. If you perform the progeny testing, there is NO guarantee that a carrier will be identified. If we perform progeny testing as a service, additional breeding time will be required. In this case, when a male and female (one pair) are identified that carry the mutation, they and their offspring will be shipped. Delivery time for strains requiring progeny testing often exceeds 25 weeks and may take 12 months or more due to the difficulties in breeding some strains. The progeny testing cost is in addition to the recovery cost and is based on the number of boxes used and the time taken to produce the mice identified as carrying the mutation.
    Please note that identified pairs may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation of the strain. Mating schemes are sometimes modified for successful cryopreservation.

    Please contact Customer Service for more information on the cost of progeny testing for a strain: Tel: 1-800-422-6423 or 1-207-288-5845 (from any location). 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* $4127.50
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 of Strains Needing Progeny Testing
    At least two untested males and two untested females (two pairs) will be recovered (eight or more mice is typical). The total number of animals provided, their gender and genotype will vary. Untested animals typically are available to ship between 13 and 16 weeks from the date of your order. If the first recovery attempt is unsuccessful, a second recovery will be done, extending the overall recovery time to approximately 25 weeks. Progeny testing is required to identify the genotype of mice of this strain, as a genotyping assay is not available. This type of testing involves breeding the recovered animals and assessing the phenotype of the offspring in order to identify animals carrying the mutation of interest. We can perform the progeny testing for you as a service or we can ship all recovered animals to you for progeny testing at your facility. If you perform the progeny testing, there is NO guarantee that a carrier will be identified. If we perform progeny testing as a service, additional breeding time will be required. In this case, when a male and female (one pair) are identified that carry the mutation, they and their offspring will be shipped. Delivery time for strains requiring progeny testing often exceeds 25 weeks and may take 12 months or more due to the difficulties in breeding some strains. The progeny testing cost is in addition to the recovery cost and is based on the number of boxes used and the time taken to produce the mice identified as carrying the mutation.
    Please note that identified pairs may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation of the strain. Mating schemes are sometimes modified for successful cryopreservation.

    Please contact Customer Service for more information on the cost of progeny testing for a strain: Tel: 1-800-422-6423 or 1-207-288-5845 (from any location). 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.

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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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