Strain Name:
CB.129S1(Cg)-Cochtm1.1Stw/Mmjax
Stock Number:
034310-JAX
Citation ID:
RRID:MMRRC_034310-JAX

Gene Information

Cochtm1.1Stw
Name: cochlin; targeted mutation 1.1, Colin L Stewart
Synonyms: Coch-
Type: Allele
Species: Mus musculus (mouse)
Chromosome: 12
Alteration at locus: Targeted Mutation
Coch
Name: cochlin
Synonyms: Coch-5B2, D12H14S564E
Type: Gene
Species: Mus musculus (mouse)
Chromosome: 12
Alteration at locus: Targeted Mutation
NCBI: 12810
VEGA: 12
HGNC: HGNC:2180
Homologene: 20868
Genotype Determination
ES Cell Line
W9.5/W95 derived from 129S1/Sv-Oca2+ Tyr+ Kitl+
Phenotype
Homozygous: 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: 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
Allelic Composition: Cochtm1.1Stw/Cochtm1.1Stw (Genetic Background: CBA/Ca.129S1-Cochtm1.1Stw )

Allelic Composition: Cochtm1.1Stw/Cochtm1.1Stw (Genetic Background: involves: 129S1/Sv )

Allelic Composition: Cochtm1.1Stw/Cochtm1.1Stw (Genetic Background: involves: 129S1/Sv * C57BL/6 * CD-1 )

Allelic Composition: Cochtm1.1Stw/Coch+ (Genetic Background: CBA/Ca.129S1-Cochtm1.1Stw )

MeSH Terms
  • Animals
  • Embryo Implantation/physiology
  • Extracellular Matrix Proteins
  • Female
  • Gene Expression Regulation/physiology
  • Genes, Reporter
  • Integrases/genetics
  • Interleukin-6/genetics
  • Lac Operon
  • Leukemia Inhibitory Factor
  • Mice
  • Mice, Inbred C3H
  • Mice, Inbred C57BL
  • Mice, Mutant Strains
  • Pregnancy
  • Protein Structure, Tertiary
  • Proteins/chemistry
  • Proteins/genetics
  • Proteins/secretion
  • RNA, Messenger/analysis
  • Up-Regulation
  • Viral Proteins/genetics
  • von Willebrand Factor/chemistry
  • Acoustic Stimulation
  • Age Factors
  • Aging
  • Analysis of Variance
  • Auditory Threshold
  • Cochlea/metabolism
  • Cochlea/pathology
  • Cochlea/physiopathology
  • Disease Models, Animal
  • Evoked Potentials, Auditory, Brain Stem
  • Gene Knock-In Techniques
  • Genotype
  • Hearing
  • Hearing Loss, Sensorineural/genetics
  • Hearing Loss, Sensorineural/metabolism
  • Hearing Loss, Sensorineural/pathology
  • Hearing Loss, Sensorineural/physiopathology
  • Mice, Inbred CBA
  • Mice, Knockout
  • Phenotype
  • Postural Balance
  • Proteins/metabolism
  • Vestibular Diseases/genetics
  • Vestibular Diseases/metabolism
  • Vestibular Diseases/pathology
  • Vestibular Diseases/physiopathology
  • Vestibular Evoked Myogenic Potentials
  • Vestibule, Labyrinth/metabolism
  • Vestibule, Labyrinth/physiopathology
  • Base Sequence
  • Brain Stem/physiopathology
  • DNA Primers
  • Hearing Loss/genetics
  • Hearing Loss/physiopathology
  • Reverse Transcriptase Polymerase Chain Reaction
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
  • Models for Human Disease
  • Neurobiology
  • Sensorineural
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

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

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

Cryopreserved material may be available upon request, please inquire to csmmrrc@jax.org for more information.

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.

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-SPERM Cryo-preserved spermatozoa $437.00 / Non-Profit Aliquot Approximate quantity3
034310-JAX-RESUS Litter recovered from cryo-archive $2,022.00 / Non-Profit Litter Recovered litter4; additional fees for any special requests.
Cryopreserved material may be available upon request, please inquire to csmmrrc@jax.org for more information.

1 The distribution fee covers the expense of rederiving mice from a live mouse; 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 contains a sufficient number of embryos (in one or more vials or straws and based on the transfer success rate of the MMRRC facility) to transfer into one to three recipients. The MMRRC makes no guarantee concerning embryo transfer success experienced in the recipient investigator's laboratory. Neither gender nor genotype ratios are guaranteed.

3 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.

4 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.

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.