Strain Name:

B6.A-Ush1gjs/J

Stock Number:

000783

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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

Former Names B6.A-Sansjs/J    (Changed: 15-DEC-04 )
B6.A-js/+    (Changed: 15-DEC-04 )
Type Congenic; Mutant Strain; Spontaneous Mutation;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Additional information on Congenic nomenclature.
Specieslaboratory mouse
Background Strain C57BL/6J
Donor Strain A/J
GenerationN18
Generation Definitions

Appearance
black, circling
Related Genotype: a/a Ush1gjs/Ush1gjs

black, unaffected
Related Genotype: a/a Ush1gjs/+

Description
Mice homozygous for the Ush1gjs allele are deaf, circle, and toss their heads from side to side. They are viable and fertile but due to their circling gait females may trample their pups and thus should not be used for breeding. No auditory brain response was detected from homozygotes at 14, 21, and 30 days of age. At 10-14 days of age the stereocilia of the outer and macular hair cells are disarrayed or missing although no abnormal phenotype is evident in inner hair cells, or in the gross anatomy of the cochlea or vestibule. The steriocilia degeneration in outer hair cells continues but the inner hair cells remain normal at 30-47 days of age. (Dickie and Deol, 1967; Kitamura et al., 1992.)

Development
The js allele of Ush1g arose spontaneously on the A/J background at The Jackson Laboratory in 1963. It was backcrossed to C57BL/6J for 17 generations, then in 1982 embryos were generated and frozen from C57BL/6J females bred with N17 heterozygous males. (Dickie and Deol, 1967.)

Control Information

  Control
   Heterozygote from the colony
 
  Considerations for Choosing Controls

Related Strains

Strains carrying other alleles of Ush1g
006111   B6(Cg)-Ush1gjs-2J/J
018420   B6.Cg-Ush1gjs-3J/Kjn
View Strains carrying other alleles of Ush1g     (2 strains)

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- Model with phenotypic similarity to human disease where etiologies involve orthologs. Human genes are associated with this disease. Orthologs of those genes appear in the mouse genotype(s).
Usher Syndrome, Type IG; USH1G
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Ush1gjs/Ush1g+

        B6.A-Ush1gjs/J
  • hearing/vestibular/ear phenotype
  • reduced linear vestibular evoked potential
    • elevated threshold and reduced amplitudes   (MGI Ref ID J:116914)

Ush1gjs/Ush1gjs

        B6.A-Ush1gjs/J
  • hearing/vestibular/ear phenotype
  • abnormal cochlear hair cell inter-stereocilial links morphology
    • lateral links that connect stereocilia to each other are sparse and frequently disrupted   (MGI Ref ID J:135991)
  • abnormal inner hair cell stereociliary bundle morphology
    • E17.5 embryos have disorganized and fragmented inner hair cell (IHC) stereociliary bundles   (MGI Ref ID J:135991)
    • stereociliary generally assemble into two to three clumps at the cell apical surface instead of forming single V-shaped bundles   (MGI Ref ID J:135991)
    • stereocilia of the small and medium rows have round, oblate tips instead of prolate tips   (MGI Ref ID J:135991)
  • abnormal orientation of cochlear hair cell stereociliary bundles
    • kinocillia show large mean deviations from the plane cell polarity axis (PCP) with only 37% of the kinocillia being within 15 degrees of the PCP compared to to 84% in wild-type mice   (MGI Ref ID J:135991)
    • the mean absolute kinociliary deviation is 25 degrees compared to 8 degrees in wild-type mice   (MGI Ref ID J:135991)
  • abnormal outer hair cell stereociliary bundle morphology
    • incomplete differentiation of stereocilia of the outer hair cells   (MGI Ref ID J:2196)
    • outer hair cells did not show regular W-shaped configuration of the stereocilia throughout the entire turns of the cochlea except for a few hair cells   (MGI Ref ID J:2196)
    • E18.5 embryos have disorganized and fragmented outer hair cell (OHC) stereociliary bundles   (MGI Ref ID J:135991)
    • stereociliary generally assemble into two to three clumps at the cell apical surface instead of forming single V-shaped bundles   (MGI Ref ID J:135991)
  • abnormal vestibular hair cell stereociliary bundle morphology
    • incomplete differentiation of stereocilia of the maculae   (MGI Ref ID J:2196)
    • in hair cells of the utricular and saccular maculae, the classical pipe organ configuration of the stereocilia was not observed   (MGI Ref ID J:2196)
  • absent linear vestibular evoked potential
    • VESPs are absent at the maximum stimulus intensity used   (MGI Ref ID J:116914)
  • cochlear outer hair cell degeneration   (MGI Ref ID J:135991)
  • deafness   (MGI Ref ID J:2196)
  • increased or absent threshold for auditory brainstem response
    • no ABR response in homozygous mice was recorded at any age   (MGI Ref ID J:2196)
  • short cochlear outer hair cells
    • many stereocillia of the small and medium rows in P0 mice are shorter than expected and do not elongate further   (MGI Ref ID J:135991)
  • behavior/neurological phenotype
  • 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)
    • abnormal pinna reflex   (MGI Ref ID J:2196)
  • circling   (MGI Ref ID J:116914)
  • head shaking   (MGI Ref ID J:2196)
  • hyperactivity   (MGI Ref ID J:2196)
  • 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)
  • nervous system phenotype
  • abnormal cochlear hair cell inter-stereocilial links morphology
    • lateral links that connect stereocilia to each other are sparse and frequently disrupted   (MGI Ref ID J:135991)
  • abnormal inner hair cell stereociliary bundle morphology
    • E17.5 embryos have disorganized and fragmented inner hair cell (IHC) stereociliary bundles   (MGI Ref ID J:135991)
    • stereociliary generally assemble into two to three clumps at the cell apical surface instead of forming single V-shaped bundles   (MGI Ref ID J:135991)
    • stereocilia of the small and medium rows have round, oblate tips instead of prolate tips   (MGI Ref ID J:135991)
  • abnormal orientation of cochlear hair cell stereociliary bundles
    • kinocillia show large mean deviations from the plane cell polarity axis (PCP) with only 37% of the kinocillia being within 15 degrees of the PCP compared to to 84% in wild-type mice   (MGI Ref ID J:135991)
    • the mean absolute kinociliary deviation is 25 degrees compared to 8 degrees in wild-type mice   (MGI Ref ID J:135991)
  • abnormal outer hair cell stereociliary bundle morphology
    • incomplete differentiation of stereocilia of the outer hair cells   (MGI Ref ID J:2196)
    • outer hair cells did not show regular W-shaped configuration of the stereocilia throughout the entire turns of the cochlea except for a few hair cells   (MGI Ref ID J:2196)
    • E18.5 embryos have disorganized and fragmented outer hair cell (OHC) stereociliary bundles   (MGI Ref ID J:135991)
    • stereociliary generally assemble into two to three clumps at the cell apical surface instead of forming single V-shaped bundles   (MGI Ref ID J:135991)
  • abnormal vestibular hair cell stereociliary bundle morphology
    • incomplete differentiation of stereocilia of the maculae   (MGI Ref ID J:2196)
    • in hair cells of the utricular and saccular maculae, the classical pipe organ configuration of the stereocilia was not observed   (MGI Ref ID J:2196)
  • cochlear outer hair cell degeneration   (MGI Ref ID J:135991)
  • short cochlear outer hair cells
    • many stereocillia of the small and medium rows in P0 mice are shorter than expected and do not elongate further   (MGI Ref ID J:135991)
View Research Applications

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

Ush1gjs related

Neurobiology Research
Hearing Defects
Vestibular Defects

Sensorineural Research
Hearing Defects
Vestibular Defects

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Ush1gjs
Allele Name Jackson shaker
Allele Type Spontaneous
Common Name(s) js;
Strain of OriginA/J
Gene Symbol and Name Ush1g, Usher syndrome 1G
Chromosome 11
Gene Common Name(s) ANKS4A; Jackson shaker; SANS; Sans; js; scaffold protein, ankyrin repeats and SAM domain containing;
Molecular Note The mutation was identified as a single bp insertion of a guanine within a stretch of six guanines at nucleotide position 675-80. The insertion causes a frame shift mutation that results in a truncated protein at amino acid position 245. The resultant protein lacks the C-terminal SAM domain. [MGI Ref ID J:82022]

Genotyping

Genotyping Information


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Additional References

Kikkawa Y; Shitara H; Wakana S; Kohara Y; Takada T; Okamoto M; Taya C; Kamiya K; Yoshikawa Y; Tokano H; Kitamura K; Shimizu K; Wakabayashi Y; Shiroishi T; Kominami R; Yonekawa H. 2003. Mutations in a new scaffold protein Sans cause deafness in Jackson shaker mice. Hum Mol Genet 12(5):453-61. [PubMed: 12588793]  [MGI Ref ID J:82022]

Kitamura K; Kakoi H; Yoshikawa Y; Ochikubo F. 1992. Ultrastructural findings in the inner ear of Jackson shaker mice. Acta Otolaryngol (Stockh) 112(4):622-7. [PubMed: 1442008]  [MGI Ref ID J:2196]

Weil D; El-Amraoui A; Masmoudi S; Mustapha M; Kikkawa Y; Laine S; Delmaghani S; Adato A; Nadifi S; Zina ZB; Hamel C; Gal A; Ayadi H; Yonekawa H; Petit C. 2003. Usher syndrome type I G (USH1G) is caused by mutations in the gene encoding SANS, a protein that associates with the USH1C protein, harmonin. Hum Mol Genet 12(5):463-71. [PubMed: 12588794]  [MGI Ref ID J:82023]

Ush1gjs related

Dickie MM; Deol MS. 1967. Jackson shaker, js Mouse News Lett 36:39.  [MGI Ref ID J:13462]

El-Amraoui A; Petit C. 2005. Usher I syndrome: unravelling the mechanisms that underlie the cohesion of the growing hair bundle in inner ear sensory cells. J Cell Sci 118(Pt 20):4593-603. [PubMed: 16219682]  [MGI Ref ID J:102194]

Grati M; Kachar B. 2011. Myosin VIIa and sans localization at stereocilia upper tip-link density implicates these Usher syndrome proteins in mechanotransduction. Proc Natl Acad Sci U S A 108(28):11476-81. [PubMed: 21709241]  [MGI Ref ID J:174394]

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]

Kikkawa Y; Shitara H; Wakana S; Kohara Y; Takada T; Okamoto M; Taya C; Kamiya K; Yoshikawa Y; Tokano H; Kitamura K; Shimizu K; Wakabayashi Y; Shiroishi T; Kominami R; Yonekawa H. 2003. Mutations in a new scaffold protein Sans cause deafness in Jackson shaker mice. Hum Mol Genet 12(5):453-61. [PubMed: 12588793]  [MGI Ref ID J:82022]

Kitamura K; Kakoi H; Yoshikawa Y; Ochikubo F. 1992. Ultrastructural findings in the inner ear of Jackson shaker mice. Acta Otolaryngol (Stockh) 112(4):622-7. [PubMed: 1442008]  [MGI Ref ID J:2196]

Lefevre G; Michel V; Weil D; Lepelletier L; Bizard E; Wolfrum U; Hardelin JP; Petit C. 2008. A core cochlear phenotype in USH1 mouse mutants implicates fibrous links of the hair bundle in its cohesion, orientation and differential growth. Development 135(8):1427-37. [PubMed: 18339676]  [MGI Ref ID J:135991]

Reiners J; Nagel-Wolfrum K; Jurgens K; Marker T; Wolfrum U. 2006. Molecular basis of human Usher syndrome: deciphering the meshes of the Usher protein network provides insights into the pathomechanisms of the Usher disease. Exp Eye Res 83(1):97-119. [PubMed: 16545802]  [MGI Ref ID J:116295]

Zheng QY; Scarborough JD; Zheng Y; Yu H; Choi D; Gillespie PG. 2012. Digenic inheritance of deafness caused by 8J allele of myosin-VIIA and mutations in other Usher I genes. Hum Mol Genet 21(11):2588-98. [PubMed: 22381527]  [MGI Ref ID J:183898]

Health & husbandry

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.

Health & Colony Maintenance Information

Animal Health Reports

Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, G200.

Colony Maintenance

Breeding & HusbandryDo not use homozygous females for breeders as they are bad mothers and often kill the litters while running around in circles.

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.

General Supply Notes

  • View the complete collection of spontaneous mutants in the Mouse Mutant Resource.

Control Information

  Control
   Heterozygote from the colony
 
  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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