These mice carry a spontaneous mutation at the Ush1c locus characterized by head tossing, hyperactive circling, and disorientation in water.
Read More +Genetic Background | Generation |
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Allele Type | Gene Symbol | Gene Name |
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Spontaneous (Modified isoform(s)) | Ush1c | USH1 protein network component harmonin |
Mice homozygous for either Ush1cdfcr or Ush1cdfcr-2J display head tossing and hyperactive circling. They are unable to orient themselves in water to swim at the surface. Despite these behavioral traits, these mice are able to breed and have normal life spans. Adult homozygotes have no auditory-evoked brainstem response. This complete deafness was found in an Ush1cdfcr homozygote as early as 21 days of age. Scanning electron micrographs of the organ of Corti at 3 weeks of age reveal disorganized and splayed stereocilia of outer hair cells and, less severely, of inner hair cells along with degeneration of hair cells that yields a disorganized hair cell pattern. While progressive loss of hair cells and a secondary loss of spiral ganglion cells are found and are severe by 8 months of age, no gross morphological abnormalities were discerned in whole mounts of inner ears. At 9 months of age a slight peripheral retinal degeneration is found in mice homozygous for Ush1cdfcr but not Ush1cdfcr-2J. These phenotypes have not been identified in mice heterozygous for either allele. (Johnson et al., 2003.)
The dfcr-2J allele of Ush1c arose spontaneously on a mixed background of C57BL/6J and 129S4 in which a targeted mutation of adducin 2 was segregating. The adducin 2 mutation has been bred out of this strain.
Allele Name | deaf circler 2 Jackson |
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Allele Type | Spontaneous (Modified isoform(s)) |
Allele Synonym(s) | |
Gene Symbol and Name | Ush1c, USH1 protein network component harmonin |
Gene Synonym(s) | |
Strain of Origin | B6;129S4-Add2tm1Llp |
Chromosome | 7 |
Molecular Note | The dfcr-2J mutation has been identified as the deletion of a single base pair, the fourth nucleotide in the inner ear-specific exon C of the 28-exon Ush1c gene. This shifts the translational reading frame shift so that 38 incorrect amino acids are incorporated before a premature stop codon is encountered. |
When using the deaf circler 2 Jackson mouse strain in a publication, please cite the originating article(s) and include JAX stock #004768 in your Materials and Methods section.
Facility Barrier Level Descriptions
Service/Product | Description | Price |
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Heterozygous for Ush1c<dfcr-2J> |
Frozen Mouse Embryo | B6;129S4-Ush1c<dfcr-2J>/J Frozen Embryos | $2595.00 |
Frozen Mouse Embryo | B6;129S4-Ush1c<dfcr-2J>/J Frozen Embryos | $2595.00 |
Frozen Mouse Embryo | B6;129S4-Ush1c<dfcr-2J>/J Frozen Embryos | $3373.50 |
Frozen Mouse Embryo | B6;129S4-Ush1c<dfcr-2J>/J Frozen Embryos | $3373.50 |
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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. We do not guarantee breeding performance and therefore suggest that investigators order more than one breeding pair to avoid delays in their research.
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