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- Description
- Disease & phenotype
- Genes & alleles
- Genotyping
- Health & care
- References
- Pricing & purchasing
- Terms of use
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. Visit our online Nomenclature tutorial. Additional information on Congenic nomenclature. Species laboratory mouse Background Strain C57BL/6J Donor Strain C57BLKS/J Generation +N3F1N1
Generation DefinitionsDescription
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
- 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)
assigned by genotype
Pcdh15av-J/Pcdh15av-J
B6.BKS-Pcdh15av-J/J
- hearing/vestibular/ear phenotype
- *normal* hearing/vestibular/ear phenotype
- abnormal cochlear hair cell morphology
- abnormal cochlear hair cell stereociliary bundle morphology
- 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)
- 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
- abnormal cochlear hair cell stereociliary bundle morphology
- 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)
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 Origin | C57BLKS/J | ||
| Gene Symbol and Name | Pcdh15, protocadherin 15 | ||
| Chromosome | 10 | ||
| Gene Common Name(s) | Ames Waltzer; BB078305; 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] | ||
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 relatedAlagramam 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 |
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Cryopreserved
Cryopreserved Mice - Ready for Recovery
Animals Provided
Price (US dollars $) Cryorecovery* $3000.00 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 (from U.S.A., Canada and Puerto Rico only) 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 |
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Cryopreserved
Cryopreserved Mice - Ready for Recovery
Animals Provided
Price (US dollars $) Cryorecovery* $3900.00 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 (from U.S.A., Canada and Puerto Rico only) 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).
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Standard Supply
Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.
General Supply Notes
- Genomic DNA is available for this strain from the Mouse DNA Resource.
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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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.
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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.Ordering Information
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