hearing/vestibular/ear
• complete detachment from the surface of the organ of Corti and association with Reissner's membrane and/or the stria vascularis
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Allele Symbol Allele Name Allele ID |
Tectatm2Gpr targeted mutation 2, Guy P Richardson MGI:3605485 |
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Summary |
2 genotypes
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• complete detachment from the surface of the organ of Corti and association with Reissner's membrane and/or the stria vascularis
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• Hensen's stripe is absent
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• limbal zone of tectorial membrane is extremely reduced, however hair bundle structure is normal
• enlargement of the subtectorial space in the vicinity of inner hair cell bundles
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• Kimura's membrane is separated from the main body of the tectorial membrane
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• the marginal band is absent
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• large holes are visible within the main body of the matrix
• striated-sheet matrix is not present in the sulcal zone and collagen fibrils are oriented almost perpendicular to its surface rather than projecting radially across the tectorial membrane
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• neural response thresholds are elevated, neural tuning is broadened, and a sharp decrease in sensitivity is observed at the tip of the neural tuning curve, however the sensitivity and frequency tuning of the mechanical responses of the cochlea are little changed
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
autosomal dominant nonsyndromic deafness 12 | DOID:0110544 |
OMIM:601543 |
J:101691 |
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO) |
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last database update 11/12/2024 MGI 6.24 |
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