Strain Detail Sheet

Strain Name    :

CB.129S1(Cg)-Cochtm1.1Stw

Stock Number :

034310-JAX

Gene Information

Gene Details [Including genotyping protocols]

(provided by MGI)
Allele Symbol: Cochtm1.1Stw
Name: targeted mutation 1.1, Colin L Stewart
Alteration at locus: Targeted Mutation
Gene Symbol: Coch
Name: coagulation factor C homolog (Limulus polyphemus)
Chromosome: 12
Alteration at locus: Targeted Mutation

Genetic Alterations:

Genotype Determination:

ES Cell Line: W9.5/W95

Strain Description [Including phenotype, strain background, strain development and suggested control mice]

Phenotype

Homozygous phenotype: Mice that are homozygous for the targeted mutation are viable, fertile and normal in size. Mice exhibit vestibular dysfunction by 13 and 21 months of age as determined by elevated measurements of the vestibular evoked potential (VsEP). Auditory brainstem response (ABR) testing at 21 months of age indicates hearing loss at the highest frequencies as determined by elevated or absent ABR thresholds. Histopathology at 21 months of age reveals no hair cell loss or obvious change in morphology. This mutant mouse strain may be useful in studies of late onset hearing loss.

Heterozygous or Hemizygous phenotype: Heterozygous mice do not have hearing loss, but exhibit the same vestibular malfunction at 21 months of age as the homozygous mice.


Mammalian Phenotype Terms:(provided by MGI)      Extend all MPTs
      assigned by genotype
Cochtm1.1Stw/Coch+
        CB.129S1-Coch<tm1.1Stw>
  • hearing/vestibular/ear phenotype
    • hearing/vestibular/ear phenotype (MGI Ref ID J:167669)
      • unlike in homozygous mutant mice, no hearing deficit is detected at any age
      • reduced linear vestibular evoked potential (MGI Ref ID J:167669)
        • increase in VsEP thresholds at 21 months of age
Cochtm1.1Stw/Cochtm1.1Stw
        CB.129S1-Coch<tm1.1Stw>
  • hearing/vestibular/ear phenotype
    • hearing/vestibular/ear phenotype (MGI Ref ID J:167669)
      • despite hearing and vestibular deficits, no gross abnormalities are seen in the cochlear duct or its accessory structures
      • absent brainstem auditory evoked potential (MGI Ref ID J:167669)
        • decreased or absent at the highest frequency test (41.2 Hz) at 21 months of age
      • decreased brainstem auditory evoked potential (MGI Ref ID J:167669)
        • decreased or absent at the highest frequency test (41.2 Hz) at 21 months of age
      • reduced linear vestibular evoked potential (MGI Ref ID J:167669)
        • increase in VsEP thresholds at 13 and 21 months of age


The following phenotype information may relate to one or more alleles on a genetic background differing from this MMRRC strain.
Cochtm1.1Stw/Cochtm1.1Stw
        involves: 129S1/Sv
  • norma l phenotype
    • no abnormal phenotype detected (MGI Ref IDs J:105566, J:108251)
      • at ~5 months, homozygotes do not display any apparent inner ear histopathology or any significant hearing loss relative to wild-type mice (MGI Ref ID J:108251)
      • surprisingly, homozygotes are viable and overtly normal and females display no defects in implantation or reproduction relative to wild-type littermates (MGI Ref ID J:105566)
Cochtm1.1Stw/Cochtm1.1Stw
        involves: 129S1/Sv * C57BL/6 * CD-1
  • hearing/vestibular/ear phenotype
    • hearing/vestibular/ear phenotype (MGI Ref ID J:116965)
      • at P19, 3- and 5- months of age, homozygotes exhibit no significant differences in auditory brainstem responses to click and pure-tone stimuli (8, 16, 32 kHz) relative to wild-type mice

Strain of Origin: 129S1/Sv-Oca1+ Tyr+ Kitl+

Strain genetic background: CB.129S1(Cg)

Strain Development: A targeting vector was designed to insert a loxP site between exons 6 and 7 and an IRESlacZ cassette followed by a loxP-flanked PGKneo cassette into exon 12. The construct was electroporated into 129S1/Sv-Oca1+ Tyr+ Kitl+ -derived W9.5 embryonic stem (ES) cells. Correctly targeted ES cells were injected into blastocysts. The resulting chimeric animals were crossed to a mixed C57BL/6 and CD1 background. Offspring were crossed with mice from a unknown background expressing Cre under the control of a beta actin promoter to remove exons 7 through 11 (containing two VWA domains) as well as the neo and lacZ cassettes and part of exon 12. Mice were subsequently crossed to CBA/CaJ for 12 generations. Upon arrival, mice were bred to CBA/CaJ for at least 1 generation to establish the colony.

Suggested Control Mice: Wild-type littermates

Research Applications

  • Models for Human Disease
  • Neurobiology
  • Sensorineural

Strain Origin

Donor: Cynthia Morton, Ph.D., Brigham and Women's Hospital, Harvard Medical School

Primary Reference:

  • Rodriguez CI; Cheng JG; Liu L; Stewart CL, Cochlin, a secreted von Willebrand factor type a domain-containing factor, is regulated by leukemia inhibitory factor in the uterus at the time of embryo implantation., Endocrinology 2004 Mar;145(3):1410-8 (Medline PMID: 14657014)
  • Jones SM; Robertson NG; Given S; Giersch AB; Liberman MC; Morton CC,Hearing and vestibular deficits in the Coch(-/-) null mouse model: Comparison to the Coch(G88E/G88E) mouse and to DFNA9 hearing and balance disorder., Hear Res. 2010 Nov 10 (Medline PMID: 21073934)
  • Makishima T; Rodriguez CI; Robertson NG; Morton CC; Stewart CL; Griffith AJ, Targeted disruption of mouse Coch provides functional evidence that DFNA9 hearing loss is not a COCH haploinsufficiency disorder., Hum Genet 2005 Oct;118(1):29-34 (Medline PMID: 16078052)

Colony and Husbandry Information

Special Considerations

While maintaining a live colony, these mice are bred as homozygotes.

Health Status Report

Mice recovered from a cryo-archive will have health surveillance performed on recipient females. Health reports will be provided prior to shipment. If you require additional health status information, please email csmmrrc@jax.org.

Order Request Information

Availability Level

Limited quantities of breeder mice (recovered litter) are available from a cryoarchive; recovered litter usually available to ship in 3 to 4 months.

Conditions of Distribution [Including applicable technology transfer agreements]

Distribution of this strain requires submission of the MMRRC Conditions of Use (COU). A link to the COU web form will be provided via email after an order has been placed; the form should be completed then or the email forwarded to your institutional official for completion.

The donor or their institution limits the distribution to non-profit institutions only.

Fees

Additional charges may apply for any special requests. Shipping costs are in addition to the basic distribution/resuscitation fees. Information on shipping costs and any additional charges will be provided by the supplying MMRRC facility.

Click button to Request this one strain. (Use the MMRRC Catalog Search to request more than one strain.)
MMRRC Item # - Description Distribution
Fee/unit (US $)
Units Notes
034310-JAX-RESUSLitter recovered from cryo-archive
$2,022.00
Non-Profit
Litter Recovered litter1; additional fees for any special requests.
034310-JAX-SPERMCryo-preserved spermatozoa
$437.00
Non-Profit
Aliquot Approximate quantity.2

1 The distribution fee covers the expense of resuscitating mice from the cryo-archive; you will receive the resulting litter. The litter will contain at minimum one mutant carrier; the actual number of animals and the gender and genotype ratios will vary. (Typically, multiple breeder pairs can be established from the recovered litter.) Prior to shipment, the MMRRC will provide information about the animals recovered. If you anticipate or find that you need to request specific genotypes, genders or quantities of mice in excess of what is likely from a resuscitated litter, you may discuss available options and pricing with the supplying MMRRC facility.

2 An aliquot is one straw or vial with sufficient sperm to recover at least one litter of mice, as per provided protocols, when performed at the MMRRC facility. The MMRRC makes no guarantee concerning the success of these procedures when performed outside the MMRRC facilities.

3 An aliquot contains a sufficient number of embryos (in one or more vials and based on the transfer success rate of the MMRRC facility) to transfer to at least two recipients. The MMRRC makes no guarantee concerning embryo transfer success experienced in the recipient investigator's laboratory. Neither gender nor genotype ratios are guaranteed.

To request material from the MMRRC: Please fill out our on-line request form (accessible from the catalog search results page, or click the Request this Strain button in the fees section above). If you have questions or need assistance completing this form, you may call Customer Service at (800) 910-2291 (in USA or Canada) or (530) 757-5710 (international calls). Before you call, please have with you: the MMRRC item number, quantity needed, Bill-to and Ship-to contact information.



To request material from the MMRRC: Please fill out our on-line request form (accessible from the catalog search results page, or click the Request this Strain button in the fees section). If you have questions or need assistance completing this form, you may call Customer Service at (800) 910-2291 (in USA or Canada) or (530) 757-5710 (international calls). Before you call, please have with you: the MMRRC item number, quantity needed, Bill-to and Ship-to contact information.



The MMRRC is a collaborative effort, funded by grants from DPCPSI of the NIH.

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