Strain Name:

B6.BKS-Pcdh15av-J/J

Stock Number:

000517

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Availability:

Cryopreserved - Ready for recovery

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; Spontaneous Mutation;
Additional information on Genetically Engineered and Mutant Mice.
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Additional information on Congenic nomenclature.
Specieslaboratory mouse
Background Strain C57BL/6J
Donor Strain C57BLKS/J
Generation+N3F1N1
Generation Definitions

Description
There have been several remutations to Ames waltzer (Pcdh15av) have occurred in stocks at The Jackson Laboratory. All mice homozygous for these remutations show the characteristic head-tossing, circling, and deafness characteristic of Ames waltzer. Ames waltzer 2J homozygous mutant mice (Pcdh15av-2J/Pcdh15av-2J) are more severely afflicted than mice homozygous for the other Jackson (J) remutations. Pcdh15av-2J/Pcdh15av-2J homozygotes become disoriented and sink in swim tests; Ames waltzer 3J homozygotes (Pcdh15av-3J/Pcdh15av-3J), like Ames waltzer-J (Pcdh15av/Pcdh15av), swim with difficulty and circle afloat. In the membranous labyrinth the fluid spaces in the Organ of Corti fail to develop. Later, hair cells and spiral ganglion cells degenerate.

Development
Ames waltzer Jackson (Pcdh15av-J) arose spontaneously in strain C57BL/Ks in 1964 at the Jackson Laboratory. An affected female was crossed to a C57BL/6J male and the stock was maintained by sibling matings using forced heterozygosis. Embryos were generated for cryopreservation in 1982 using heterozygous males at F37N3 bred with C57BL/6J females.

Control Information

  Control
   Untyped from the colony
   000664 C57BL/6J
 
  Considerations for Choosing Controls

Related Strains

Strains carrying other alleles of Pcdh15
002078   B6C3Fe a/a-Pcdh15av-2J/J
002072   C57BL/6J-Pcdh15av-3J/J
004156   C57BL/6J-Pcdh15av-5J/J
View Strains carrying other alleles of Pcdh15     (3 strains)

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- Potential model based on gene homology relationships. Phenotypic similarity to the human disease has not been tested.
Deafness, Autosomal Recessive 23; DFNB23   (PCDH15)
Usher Syndrome, Type ID; USH1D   (PCDH15)
Usher Syndrome, Type IF; USH1F   (PCDH15)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Pcdh15av-J/Pcdh15av-J

        B6.BKS-Pcdh15av-J/J
  • hearing/vestibular/ear phenotype
  • *normal* hearing/vestibular/ear phenotype
    • normal endocochlear potential   (MGI Ref ID J:106625)
    • histochemistry of the organ of Corti shows the centrosomes in the normal position   (MGI Ref ID J:106625)
    • cytocauds are not observed   (MGI Ref ID J:106625)
    • abnormal cochlear hair cell morphology
      • diminished amounts of actin in the stereocilla and cuticular plate, distorted shape of the outer perimeter of all hair cells   (MGI Ref ID J:106625)
      • inner hair cells more severely affected than outer hair cells   (MGI Ref ID J:106625)
      • abnormal cochlear hair cell stereociliary bundle morphology
        • at 16 days of age stereocilia bundles are disorganized and some are clumped together; the angle between the stereocilia and the reticular lamina is abnormal   (MGI Ref ID J:106625)
        • the luminal surface of Deiters cells has more microvilli than normal   (MGI Ref ID J:106625)
        • abnormal inner hair cell stereociliary bundle morphology
          • inner hair cell stereocilia at 16 days of age are disorganized and appear to be bent at a variety of angles   (MGI Ref ID J:106625)
          • abnormal orientation of inner hair cell stereociliary bundles   (MGI Ref ID J:106625)
        • short cochlear hair cell stereocilia   (MGI Ref ID J:106625)
      • abnormal cochlear outer hair cell morphology
        • at 16 days of age outer hair cell shape is normal, but the number of mitochondria is low and the nucleus is darker than normal with early signs of DNA clumping   (MGI Ref ID J:106625)
      • cochlear hair cell degeneration   (MGI Ref ID J:106625)
    • abnormal organ of Corti supporting cell morphology
      • diminished actin in the supporting cells, particularly the pillar and Hensen cells   (MGI Ref ID J:106625)
      • abnormal Hensen cell morphology   (MGI Ref ID J:106625)
      • abnormal pillar cell morphology   (MGI Ref ID J:106625)
    • absent linear vestibular evoked potential
      • VESPs are absent at the maximum stimulus intensity used   (MGI Ref ID J:116914)
    • increased or absent threshold for auditory brainstem response
      • as early as 9 days of age there is no ABR response even at 20 kHz   (MGI Ref ID J:106625)
  • behavior/neurological phenotype
  • circling   (MGI Ref ID J:106625)
  • impaired swimming   (MGI Ref ID J:106625)
    • 7 of 10 mice tested exhibit poor swimming ability; mice can not maneuver in the water and can not remain at the surface   (MGI Ref ID J:116914)
    • 3 of 10 mice tested exhibit reduced swimming ability; mice can swim at or near the surface of the water, but do not maintain a horizontal body position   (MGI Ref ID J:116914)
  • nervous system phenotype
  • abnormal cochlear hair cell morphology
    • diminished amounts of actin in the stereocilla and cuticular plate, distorted shape of the outer perimeter of all hair cells   (MGI Ref ID J:106625)
    • inner hair cells more severely affected than outer hair cells   (MGI Ref ID J:106625)
    • abnormal cochlear hair cell stereociliary bundle morphology
      • at 16 days of age stereocilia bundles are disorganized and some are clumped together; the angle between the stereocilia and the reticular lamina is abnormal   (MGI Ref ID J:106625)
      • the luminal surface of Deiters cells has more microvilli than normal   (MGI Ref ID J:106625)
      • abnormal inner hair cell stereociliary bundle morphology
        • inner hair cell stereocilia at 16 days of age are disorganized and appear to be bent at a variety of angles   (MGI Ref ID J:106625)
        • abnormal orientation of inner hair cell stereociliary bundles   (MGI Ref ID J:106625)
      • short cochlear hair cell stereocilia   (MGI Ref ID J:106625)
    • abnormal cochlear outer hair cell morphology
      • at 16 days of age outer hair cell shape is normal, but the number of mitochondria is low and the nucleus is darker than normal with early signs of DNA clumping   (MGI Ref ID J:106625)
    • cochlear hair cell degeneration   (MGI Ref ID J:106625)
View Research Applications

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

Pcdh15av-J related

Neurobiology Research
Hearing Defects

Sensorineural Research
Hearing Defects

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Pcdh15av-J
Allele Name Ames waltzer Jackson
Allele Type Spontaneous
Common Name(s) avJ;
Strain of OriginC57BLKS/J
Gene Symbol and Name Pcdh15, protocadherin 15
Chromosome 10
Gene Common Name(s) Ames Waltzer; BB078305; CDHR15; DFNB23; ENSMUSG00000046980; Gm9815; USH1F; av; expressed sequence BB078305; neuroscience mutagenesis center, 19; neuroscience mutagenesis facility, 19; nmf19; predicted gene 9815; predicted gene, ENSMUSG00000046980;
General Note Several remutations to Pcdh15 have occurred in stocks at The Jackson Laboratory. All show the characteristic head-tossing, circling, and deafness (J:28688).
Molecular Note The mutation is a 834 bp deletion that is predicted to delete 278 amino acids from the extracellular domain of the encoded protein. [MGI Ref ID J:66738]

Genotyping

Genotyping Information


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Additional References

Alagramam KN; Murcia CL; Kwon HY; Pawlowski KS; Wright CG; Woychik RP. 2001. The mouse ames waltzer hearing-loss mutant is caused by mutation of pcdh15, a novel protocadherin gene Nat Genet 27(1):99-102. [PubMed: 11138007]  [MGI Ref ID J:66738]

Osako S; Hilding DA. 1971. Electron microscopic studies of capillary permeability in normal and ames waltzer deaf mice. Acta Otolaryngol (Stockh) 71(5):365-76. [PubMed: 5093631]  [MGI Ref ID J:5225]

Pcdh15av-J related

Alagramam KN; Murcia CL; Kwon HY; Pawlowski KS; Wright CG; Woychik RP. 2001. The mouse ames waltzer hearing-loss mutant is caused by mutation of pcdh15, a novel protocadherin gene Nat Genet 27(1):99-102. [PubMed: 11138007]  [MGI Ref ID J:66738]

Alagramam KN; Stahl JS; Jones SM; Pawlowski KS; Wright CG. 2005. Characterization of vestibular dysfunction in the mouse model for Usher syndrome 1F. J Assoc Res Otolaryngol 6(2):106-18. [PubMed: 15952048]  [MGI Ref ID J:148677]

Alagramam KN; Zahorsky-Reeves J; Wright CG; Pawlowski KS; Erway LC; Stubbs L; Woychik RP. 2000. Neuroepithelial defects of the inner ear in a new allele of the mouse mutation Ames waltzer. Hear Res 148(1-2):181-91. [PubMed: 10978835]  [MGI Ref ID J:66637]

Ball SL; Bardenstein D; Alagramam KN. 2003. Assessment of retinal structure and function in Ames waltzer mice. Invest Ophthalmol Vis Sci 44(9):3986-92. [PubMed: 12939319]  [MGI Ref ID J:85211]

Cook S; Lane P. 1993. Re-mutation to Ames waltzer Mouse Genome 91:554.  [MGI Ref ID J:28688]

Jones SM; Johnson KR; Yu H; Erway LC; Alagramam KN; Pollak N; Jones TA. 2005. A quantitative survey of gravity receptor function in mutant mouse strains. J Assoc Res Otolaryngol 6(4):297-310. [PubMed: 16235133]  [MGI Ref ID J:116914]

Pawlowski KS; Kikkawa YS; Wright CG; Alagramam KN. 2006. Progression of inner ear pathology in Ames waltzer mice and the role of protocadherin 15 in hair cell development. J Assoc Res Otolaryngol 7(2):83-94. [PubMed: 16408167]  [MGI Ref ID J:148675]

Raphael Y; Kobayashi KN; Dootz GA; Beyer LA; Dolan DF; Burmeister M. 2001. Severe vestibular and auditory impairment in three alleles of Ames waltzer (av) mice. Hear Res 151(1-2):237-249. [PubMed: 11124469]  [MGI Ref ID J:106625]

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.

Control Information

  Control
   Untyped 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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