Strain Name:

CBA.129S-Tectatm1.1Ogha/J

Stock Number:

010826

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Availability:

Cryopreserved - Ready for recovery

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This targeted mutation of the tectorin alpha (Tecta) gene displays partial to severe hearing loss and may be useful in studies of cochlear physiology and congenital hearing loss.

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 CB.129S-Tectatm1.1Ogha/J    (Changed: 31-JUL-12 )
Type Congenic; Mutant Strain; Targeted Mutation;
Additional information on Genetically Engineered and Mutant Mice.
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Additional information on Congenic nomenclature.
Specieslaboratory mouse
 
Donating Investigator John S Oghalai,   Baylor College of Medicine

Description
Mice that are homozygous for the targeted mutation are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. Heterozygous mice have a thickened and shortened tectorial membrane, which covers only the first of the three rows of the cochlear outer hair cells. In homozygotes the membrane is thicker, appears to be detached from the sensory epithelium, loosely connected to the spiral limbus and elevated from the organ of Corti. Heterozygous and homozygous mice exhibit, respectively, reduced and absent forward transduction, partial and severe hearing loss, partial and severe disruption of the fibrils surrounding the edges of the tectorial membrane. Both genotypes exhibit enhanced reverse transduction.

Development
The targeting vector was designed to change the fourth von Willebrand Factor domain of the murine Tecta cDNA sequence (exons 13 and 14) from a cysteine to glycine at amino acid position 1509 (C1509G) of exon 14. An FRT-flanked neomycin cassette was inserted upstream of the coding sequence. The construct was electroporated into 129S7/SvEvBrd derived AB2.1 embryonic stem (ES) cells. Correctly targeted ES cells were injected into blastocysts. The resulting chimeric animals were crossed to 129S6/SvEv and the F1 progeny crossed to 129S4/SvJaeSor-Gt(ROSA)26Sortm1(FLP1)Dym/J to remove the neo cassette. Heterozygotes were crossed to CBA for five generations in the laboratory of the donating investigator. Upon arrival, mice were bred to CBA/J for at least 1 generation to establish the colony.

Control Information

  Control
   000656 CBA/J
 
  Considerations for Choosing Controls

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 Dominant 12; DFNA12   (TECTA)
Deafness, Autosomal Recessive 21; DFNB21   (TECTA)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

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

Tectatm1.1Ogha/Tecta+

        involves: 129S4/SvJaeSor * 129S7/SvEvBrd * CBA
  • hearing/vestibular/ear phenotype
  • abnormal cochlear outer hair cell physiology
    • during acoustic stimulation of the stapes, only outer hair cells in the first row exhibit forward transduction unlike in similarly treated wild-type cells   (MGI Ref ID J:160851)
    • abnormal cochlear microphonics
      • in response to an increasing intensity of accoustic stimuli, mice exhibit an increase in cochlear microphonic phase lead unlike in similarly treated wild-type mice   (MGI Ref ID J:160851)
  • abnormal otoacoustic response
    • mice exhibit enhanced reverse transduction compared with wild-type mice   (MGI Ref ID J:160851)
    • abnormal distortion product otoacoustic emission
      • distortion product otoacoustic emission exhibit elevated thresholds, reduced amplitudes, and higher slopes compared to in wild-type mice   (MGI Ref ID J:160851)
  • abnormal tectorial membrane morphology
    • the tectorial membrane is thicker and shorter than in wild-type mice but not as much as in homozygous mice   (MGI Ref ID J:160851)
    • the fibrils surrounding the edge of the tectorial membrane are partially disrupted compared to in wild-type mice   (MGI Ref ID J:160851)
    • Kimura's membrane contains loosely packed fibrils compared to in wild-type mice   (MGI Ref ID J:160851)
  • impaired hearing
    • mice exhibit partial hearing loss compared with wild-type mice   (MGI Ref ID J:160851)
  • increased or absent threshold for auditory brainstem response
    • heterozygous mice had 25-40 dB ABR threshold elevations relative to wild-type littermates   (MGI Ref ID J:160851)
  • nervous system phenotype
  • abnormal cochlear outer hair cell physiology
    • during acoustic stimulation of the stapes, only outer hair cells in the first row exhibit forward transduction unlike in similarly treated wild-type cells   (MGI Ref ID J:160851)
    • abnormal cochlear microphonics
      • in response to an increasing intensity of accoustic stimuli, mice exhibit an increase in cochlear microphonic phase lead unlike in similarly treated wild-type mice   (MGI Ref ID J:160851)

Tectatm1.1Ogha/Tectatm1.1Ogha

        involves: 129S4/SvJaeSor * 129S7/SvEvBrd * CBA
  • hearing/vestibular/ear phenotype
  • *normal* hearing/vestibular/ear phenotype
    • mice exhibit normal vestibular function   (MGI Ref ID J:160851)
    • abnormal cochlear outer hair cell physiology
      • during acoustic stimulation of the stapes, outer hair cells in all rows exhibit no forward transduction unlike in similarly treated wild-type cells   (MGI Ref ID J:160851)
      • decreased cochlear microphonics
        • in response to an accoustic stimuli, cochlear microphonic is asymmetrical, reduced in amplitude, and a quarter of a cycle ahead of the stimulus unlike in wild-type mice   (MGI Ref ID J:160851)
    • abnormal tectorial membrane morphology
      • the tectorial membrane is thicker and shorter than in wild-type and heterozygous mice   (MGI Ref ID J:160851)
      • the shortened tectorial membrane does not cover all three rows of the outer hair cells unlike in wild-type mice   (MGI Ref ID J:160851)
      • the fibrils surrounding the edge of the tectorial membrane are severely disrupted compared to in wild-type mice   (MGI Ref ID J:160851)
      • Kimura's membrane contains severely disrupted fibrils compared to in wild-type mice   (MGI Ref ID J:160851)
      • detached tectorial membrane   (MGI Ref ID J:160851)
    • absent distortion product otoacoustic emissions   (MGI Ref ID J:160851)
    • impaired hearing
      • mice exhibit severe hearing loss compared with wild-type mice   (MGI Ref ID J:160851)
    • increased or absent threshold for auditory brainstem response
      • homozygous mice had 30-50 dB ABR threshold elevations relative to wild-type littermates   (MGI Ref ID J:160851)
  • nervous system phenotype
  • abnormal cochlear outer hair cell physiology
    • during acoustic stimulation of the stapes, outer hair cells in all rows exhibit no forward transduction unlike in similarly treated wild-type cells   (MGI Ref ID J:160851)
    • decreased cochlear microphonics
      • in response to an accoustic stimuli, cochlear microphonic is asymmetrical, reduced in amplitude, and a quarter of a cycle ahead of the stimulus unlike in wild-type mice   (MGI Ref ID J:160851)

Tectatm1.1Ogha/Tectatm1.1Ogha

        involves: 129S7/SvEvBrd * CBA
  • hearing/vestibular/ear phenotype
  • abnormal otoacoustic response
    • electrically evoked otoacoustic emissions amplitudes are increased compared to in wild-type mice   (MGI Ref ID J:160851)
View Research Applications

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

Neurobiology Research
Hearing Defects

Sensorineural Research
Hearing Defects

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Tectatm1.1Ogha
Allele Name targeted mutation 1.1, John Oghalai
Allele Type Targeted
Common Name(s) TectaC1509G;
Mutation Made By John Oghalai,   Baylor College of Medicine
Strain of Origin129S7/SvEvBrd-Hprt
Promoter Tecta, tectorin alpha, mouse, laboratory
Molecular Note Exon 14 was replaced with a modified one in which nucleotide substitution (T to G) resulted in the amino acid substitution of glycine for cysteine at position 1509 (C1509G). This mutation was identified in human patients with autosomal dominant hearing loss. A second silent nucleotide substitution (C to A) at codon for amino acid 1510 was generated to assist in genotypying. A frt flanked neo cassette was inserted upstream of exon 14 and was removed by flp mediated recombination. [MGI Ref ID J:160851]

Genotyping

Genotyping Information


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Additional References

Tectatm1.1Ogha related

Xia A; Gao SS; Yuan T; Osborn A; Bress A; Pfister M; Maricich SM; Pereira FA; Oghalai JS. 2010. Deficient forward transduction and enhanced reverse transduction in the alpha tectorin C1509G human hearing loss mutation. Dis Model Mech 3(3-4):209-23. [PubMed: 20142329]  [MGI Ref ID J:160851]

Xia A; Song Y; Wang R; Gao SS; Clifton W; Raphael P; Chao SI; Pereira FA; Groves AK; Oghalai JS. 2013. Prestin regulation and function in residual outer hair cells after noise-induced hearing loss. PLoS One 8(12):e82602. [PubMed: 24376553]  [MGI Ref ID J:211128]

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 & HusbandryWhile maintaining a live colony, these mice are bred as heterozygotes or homozygotes.

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 will fulfill 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 will fulfill 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.

Control Information

  Control
   000656 CBA/J
 
  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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