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Strain Name:

B6.A-Ush1gjs/J

Stock Number:

000783

Availability:

Repository- Live

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General Terms and Conditions

Former Name      B6.A-Sansjs/J    (Changed: 15-DEC-04 )
      B6.A-js/+    (Changed: 15-DEC-04 )
Genes & Alleles   Ush1g;   Ush1gjs;

Product Information

Strain Details

Type JAX® GEMM® Strain - Congenic
Additional information on JAX® GEMM® Strains.
Type JAX® GEMM® Strain - Mutant Strain
Type JAX® GEMM® Strain - Spontaneous Mutation
Mating SystemHeterozygote x Homozygote         (Female x Male)
Specieslaboratory mouse
Background Strain C57BL/6J
Donor Strain A/J
Generation[N18p]+N2F13 (06-DEC-07)

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

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

Strain 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.)

Strain 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.)

Mammalian Phenotype Terms assigned by genotype

Ush1gjs/Ush1g+

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

Ush1gjs/Ush1gjs

        B6.A-Ush1gjs/J
  • hearing/vestibular/ear phenotype
  • abnormal cochlear hair cell inter-stereocilial links (MGI Ref ID J:135991)
    • lateral links that connect stereocilia to each other are sparse and frequently disrupted
  • abnormal inner hair cell stereociliary bundle morphology (MGI Ref ID J:135991)
    • E17.5 embryos have disorganized and fragmented inner hair cell (IHC) stereociliary bundles
    • stereociliary generally assemble into two to three clumps at the cell apical surface instead of forming single V-shaped bundles
    • stereocilia of the small and medium rows have round, oblate tips instead of prolate tips
  • abnormal orientation of cochlear hair cell stereociliary bundles (MGI Ref ID J:135991)
    • 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
    • the mean absolute kinociliary deviation is 25 degrees compared to 8 degrees in wild-type mice
  • abnormal outer hair cell stereociliary bundle morphology (MGI Ref ID J:2196)
    • incomplete differentiation of stereocilia of the outer hair cells
    • 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
    • E18.5 embryos have disorganized and fragmented outer hair cell (OHC) stereociliary bundles
    • stereociliary generally assemble into two to three clumps at the cell apical surface instead of forming single V-shaped bundles
  • abnormal pinna reflex (MGI Ref ID J:2196)
  • abnormal vestibular hair cell stereociliary bundle morphology (MGI Ref ID J:2196)
    • incomplete differentiation of stereocilia of the maculae
    • in hair cells of the utricular and saccular maculae, the classical pipe organ configuration of the stereocilia was not observed
  • absent brainstem auditory evoked potential (MGI Ref ID J:2196)
    • no ABR response in homozygous mice was recorded at any age
  • absent linear vestibular evoked potential (MGI Ref ID J:116914)
    • VESPs are absent at the maximum stimulus intensity used
  • circling (MGI Ref ID J:116914)
  • cochlear outer hair cell degeneration (MGI Ref ID J:135991)
  • deafness (MGI Ref ID J:2196)
  • head shaking (MGI Ref ID J:2196)
  • short cochlear outer hair cells (MGI Ref ID J:135991)
    • many stereocillia of the small and medium rows in P0 mice are shorter than expected and do not elongate further
  • behavior/neurological phenotype
  • abnormal reflex (MGI Ref ID J:116914)
    • 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
    • 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 (MGI Ref ID J:116914)
    • mice exhibit poor swimming ability; mice can not maneuver in the water and can not remain at the surface
  • nervous system phenotype
  • abnormal cochlear hair cell inter-stereocilial links (MGI Ref ID J:135991)
    • lateral links that connect stereocilia to each other are sparse and frequently disrupted
  • abnormal inner hair cell stereociliary bundle morphology (MGI Ref ID J:135991)
    • E17.5 embryos have disorganized and fragmented inner hair cell (IHC) stereociliary bundles
    • stereociliary generally assemble into two to three clumps at the cell apical surface instead of forming single V-shaped bundles
    • stereocilia of the small and medium rows have round, oblate tips instead of prolate tips
  • abnormal orientation of cochlear hair cell stereociliary bundles (MGI Ref ID J:135991)
    • 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
    • the mean absolute kinociliary deviation is 25 degrees compared to 8 degrees in wild-type mice
  • abnormal outer hair cell stereociliary bundle morphology (MGI Ref ID J:2196)
    • incomplete differentiation of stereocilia of the outer hair cells
    • 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
    • E18.5 embryos have disorganized and fragmented outer hair cell (OHC) stereociliary bundles
    • stereociliary generally assemble into two to three clumps at the cell apical surface instead of forming single V-shaped bundles
  • abnormal vestibular hair cell stereociliary bundle morphology (MGI Ref ID J:2196)
    • incomplete differentiation of stereocilia of the maculae
    • in hair cells of the utricular and saccular maculae, the classical pipe organ configuration of the stereocilia was not observed
  • cochlear outer hair cell degeneration (MGI Ref ID J:135991)
  • short cochlear outer hair cells (MGI Ref ID J:135991)
    • many stereocillia of the small and medium rows in P0 mice are shorter than expected and do not elongate further

Gene & Allele Details

Allele Symbol Ush1gjs
Allele Name Jackson shaker
Common Name(s) js;
Strain of OriginA/J
Gene Symbol and Name Ush1g, Usher syndrome 1G homolog (human)
Chromosome 11
Gene Common Name(s) ANKS4A; FLJ33924; 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]

Control Information

  Allele   Control
 Ush1gjs  Heterozygote from the colony
 
  Considerations for Choosing Controls

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.

Related Strains

Strains carrying other alleles of Ush1g
006111   B6(Cg)-Ush1gjs-2J/J
View Strains carrying other alleles of Ush1g     (1 strain)

Additional Web Information

Congenic Nomenclature

Animal Health Reports

Room Number           A1

Research Applications

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

Ush1gjs related

Neurobiology Research
Vestibular and Hearing Defects

Sensorineural Research
Vestibular and Hearing Defects

References

Additional References

Price and Supply Information

Strain Name: B6.A-Ush1gjs/J
Stock Number: 000783

Price Details

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Supply Details

Standard SupplyRepository-Live. A collection of over 1000 strains maintained as live colonies. Individual colonies are sized to meet current customer demand. Delivery for orders of 10 mice or less ranges on average from one to eight weeks; mice are generally shipped between four to six weeks of age with a maximum shipping age of ~nine weeks. Colony sizes do not generally support stringent age specifications for large volumes of mice; however custom orders and larger quantities of mice are easily arranged. Estimated ship dates for all orders provided within 48 hours of order placement.
Supply Notes Usually shipped between four and eight weeks of age.
This strain is included in the Mouse Mutant Resource collection.
Genomic DNA is available for this strain from the Mouse DNA Resource.
LicensingSee General Terms and Conditions below  
Control InformationView Control Information in Strain Details.

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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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