Strain Name:

CByJ.A-Atp2b2dfw-2J/J

Stock Number:

002894

Order this mouse

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

Former Names CByJ.A-Ttc7+? Atp2b2dfw-2J/J    (Changed: 03-AUG-06 )
CByJ.A-fsn+? Atp2b2dfw-2J/J    (Changed: 15-APR-05 )
CBy.A-fsn+? Atp2b2dfw-2J/J    (Changed: 15-DEC-04 )
Type Congenic; Mutant Strain;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Additional information on Congenic nomenclature.
Specieslaboratory mouse

Appearance
albino, ataxic
Related Genotype: A/A Tyrp1b/Tyrp1b Tyrc/Tycc Atp2b2dfw-2J/Atp2b2dfw-2J

albino, unaffected
Related Genotype: A/A Tyrp1b/Tyrp1b Tyrc/Tyrc Atp2b2dfw-2J/+ or A/A Tyrp1b/Tyrp1b Tyrc/Tyrc +/+

Description
The deaf waddler 2J allele (Atp2b2dfw-2J) arose spontaneously in a congenic substrain of BALB/cByJ mice, CByJ.A-fsn/J. Homozygous mutant mice are viable, but neither sex will breed. They are recognizable by 12-14 days of age by their hesitant, wobbly gait and head bobbing behavior. Homozygotes also develop a slight body tremor and become increasingly lethargic. Homozygous mutant mice are profoundly deaf, exhibiting no discernible auditory brainstem responses (ABR) to stimuli up to 100 dB. Heterozygous Atp2b2dfw-2J mutant mice also show age-dependent changes in ABR and progressive hearing loss as a result of a modifier allele (ahl) present in BALB/cByJ mice.

Control Information

  Control
   Heterozygote from the colony
   Wild-type from the colony
   001026 BALB/cByJ
 
  Considerations for Choosing Controls

Related Strains

Strains carrying other alleles of Atp2b2
016172   B6(C3)-Atp2b2Lilo/JtJ
003892   B6;BKS-Atp2b2dfw-3J/J
001276   C3H/HeJ-Atp2b2dfw/J
013746   STOCK Atp2b2tm1Ges/Mmjax
View Strains carrying other alleles of Atp2b2     (4 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 12; DFNB12   (ATP2B2)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Atp2b2dfw-2J/Atp2b2+

        CByJ.A-Atp2b2dfw-2J/J
  • hearing/vestibular/ear phenotype
  • deafness
    • show age-dependent hearing loss by 12 weeks of age   (MGI Ref ID J:38429)
  • increased or absent threshold for auditory brainstem response
    • at 4 to 5 weeks of age, significantly elevated sound pressure thresholds   (MGI Ref ID J:38429)
  • increased susceptibility to age-related hearing loss
    • hearing loss begins 4 weeks after birth and progresses rapidly to complete deafness by 12 weeks of age   (MGI Ref ID J:38429)
  • reduced linear vestibular evoked potential
    • elevated threshold and reduced amplitudes   (MGI Ref ID J:116914)

Atp2b2dfw-2J/Atp2b2dfw-2J

        CByJ.A-Atp2b2dfw-2J/J
  • hearing/vestibular/ear phenotype
  • absent linear vestibular evoked potential
    • VESPs are absent at the maximum stimulus intensity used   (MGI Ref ID J:116914)
  • deafness
    • profoundly deaf   (MGI Ref ID J:38429)
    • complete deafness at 3 weeks after birth   (MGI Ref ID J:38429)
  • increased or absent threshold for auditory brainstem response
    • no discernible auditory brainstem responses (ABR) to stimuli up to 100 dB   (MGI Ref ID J:38429)
  • behavior/neurological phenotype
  • abnormal gait
    • walk with a hesitant and wobbly gait   (MGI Ref ID J:38429)
  • abnormal reflex
    • abnormal drop reflex; mice do not demonstrate expected dorsoflexion and spread out the front paws when quickly lowered from ~20 cm above a table surface, while controls do exhibit this behavior   (MGI Ref ID J:116914)
  • ataxia
    • highly unbalanced and uncontrolled movements starting by 12-14 days of age   (MGI Ref ID J:38429)
  • head bobbing   (MGI Ref ID J:38429)
  • impaired swimming
    • mice exhibit poor swimming ability; mice can not maneuver in the water and can not remain at the surface   (MGI Ref ID J:116914)
  • lethargy
    • becomes increasingly lethargic   (MGI Ref ID J:38429)
  • tremors
    • a slight body tremor   (MGI Ref ID J:38429)
  • reproductive system phenotype
  • infertility
    • life span is normal, but neither sex breeds   (MGI Ref ID J:38429)

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

Atp2b2dfw-2J/Atp2b2+

        involves: CAST/Ei * BALB/cByJ
  • hearing/vestibular/ear phenotype
  • *normal* hearing/vestibular/ear phenotype   (MGI Ref ID J:70964)
    • in a mapping cross heterozygosity for deaf waddler 2 Jackson is found to have normal hearing when the age related hearing loss allele is absent proving this to be the recessive deafness-enhancing modifier, which is absent in CAST/Ei   (MGI Ref ID J:38429)
View Research Applications

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

Atp2b2dfw-2J related

Cell Biology Research
Channel and Transporter Defects
      calcium

Neurobiology Research
Ataxia (Movement) Defects
Channel and Transporter Defects
      calcium
Hearing Defects
Vestibular Defects

Sensorineural Research
Hearing Defects
Vestibular Defects

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Atp2b2dfw-2J
Allele Name deaf waddler 2 Jackson
Allele Type Spontaneous
Common Name(s) dfw-2J;
Strain of OriginCByJ.A-Ttc7fsn/J
Gene Symbol and Name Atp2b2, ATPase, Ca++ transporting, plasma membrane 2
Chromosome 6
Gene Common Name(s) D6Abb2e; DNA segment, Chr 6, Abbott 2e; Gena300; Genetics Harwell, 300; PMCA2; PMCA2a; PMCA2i; Tmy; deaf waddler; dfw; jog; joggle; tommy; wms; wri; wriggle; wriggle mouse Sagami;
Molecular Note A 2 bp nucleotide deletion at positions 1368 and 1369 cause a frameshift that results in a premature stop and truncation of the encoded protein at position 471. [MGI Ref ID J:48969]

Genotyping

Genotyping Information

Genotyping Protocols

Atp2b2dfw-2J, Pyrosequencing
Atp2b2dfw-2J, End Point Analysis


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Street VA; McKee-Johnson JW; Fonseca RC; Tempel BL; Noben-Trauth K. 1998. Mutations in a plasma membrane Ca2+-ATPase gene cause deafness in deafwaddler mice. Nat Genet 19(4):390-4. [PubMed: 9697703]  [MGI Ref ID J:48969]

Additional References

McCullough BJ; L Tempel B. 2004. Haplo-insufficiency revealed in deafwaddler mice when tested for hearing loss and ataxia. Hear Res 195(1-2):90-102. [PubMed: 15350283]  [MGI Ref ID J:92390]

Noben-Trauth K; Zheng QY; Johnson KR; Nishina PM. 1997. mdfw: a deafness susceptibility locus that interacts with deaf waddler (dfw). Genomics 44(3):266-72. [PubMed: 9325047]  [MGI Ref ID J:38429]

Atp2b2dfw-2J related

Duncan JL; Yang H; Doan T; Silverstein RS; Murphy GJ; Nune G; Liu X; Copenhagen D; Tempel BL; Rieke F; Krizaj D. 2006. Scotopic visual signaling in the mouse retina is modulated by high-affinity plasma membrane calcium extrusion. J Neurosci 26(27):7201-11. [PubMed: 16822977]  [MGI Ref ID J:110230]

Johnson KR; Zheng QY; Noben-Trauth K. 2006. Strain background effects and genetic modifiers of hearing in mice. Brain Res 1091(1):79-88. [PubMed: 16579977]  [MGI Ref ID J:110459]

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]

Kurnellas MP; Nicot A; Shull GE; Elkabes S. 2005. Plasma membrane calcium ATPase deficiency causes neuronal pathology in the spinal cord: a potential mechanism for neurodegeneration in multiple sclerosis and spinal cord injury. FASEB J 19(2):298-300. [PubMed: 15576480]  [MGI Ref ID J:105109]

McCullough BJ; L Tempel B. 2004. Haplo-insufficiency revealed in deafwaddler mice when tested for hearing loss and ataxia. Hear Res 195(1-2):90-102. [PubMed: 15350283]  [MGI Ref ID J:92390]

Noben-Trauth K; Zheng QY; Johnson KR; Nishina PM. 1997. mdfw: a deafness susceptibility locus that interacts with deaf waddler (dfw). Genomics 44(3):266-72. [PubMed: 9325047]  [MGI Ref ID J:38429]

VanHouten J; Sullivan C; Bazinet C; Ryoo T; Camp R; Rimm DL; Chung G; Wysolmerski J. 2010. PMCA2 regulates apoptosis during mammary gland involution and predicts outcome in breast cancer. Proc Natl Acad Sci U S A 107(25):11405-10. [PubMed: 20534448]  [MGI Ref ID J:161282]

VanHouten JN; Neville MC; Wysolmerski JJ. 2007. The calcium-sensing receptor regulates plasma membrane calcium adenosine triphosphatase isoform 2 activity in mammary epithelial cells: a mechanism for calcium-regulated calcium transport into milk. Endocrinology 148(12):5943-54. [PubMed: 17823248]  [MGI Ref ID J:131135]

Zheng QY; Johnson KR. 2001. Hearing loss associated with the modifier of deaf waddler (mdfw) locus corresponds with age-related hearing loss in 12 inbred strains of mice. Hear Res 154(1-2):45-53. [PubMed: 11423214]  [MGI Ref ID J:70964]

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.

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Supply Notes

  • 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 willfulfill 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.

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Supply Notes

  • 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 willfulfill 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
   Heterozygote from the colony
   Wild-type from the colony
   001026 BALB/cByJ
 
  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.


(6.6)