hearing/vestibular/ear
IMPC - HMGU
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hematopoietic system
IMPC - HMGU
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Allele Symbol Allele Name Allele ID |
Rest+ wild type MGI:2433490 |
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Summary |
10 genotypes
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Data Sources
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
IMPC - HMGU
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IMPC - HMGU
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• kinocilia are abnormally short in the utricles at E15 and E16
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• most outer hair cells lack stereocilia at P6
• organ of Corti cultures from P1 mice show degeneration of outer hair cell stereocilia
• organ of Corti cultures from P1 mice treated with the HDAC inhibitors FK228, SAHA, or Merck60 show prevention of outer hair cell stereocilia degeneration
• mice injected with the HDAC inhibitor SAHA at P2, P4, and P5 show retention of stereocilia in outer hair cells
• SAHA treatment is more effective at rescuing the outer hair cells at P6 than at P16
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• hair cells are present until E16-17 but degenerate throughout the inner ear by P12
• the hearing organ of P6 mice shows increased apoptosis in hair cells
• the caspase inhibitors Z-DEVD-FMK and Z-VAD-FMK delay the death of hair cells in organ of Corti cultures but do not prevent the degeneration of stereocilia
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• cilia are abnormally short in the utricles at E15 and E16
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• mice exhibit balance defects
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• kinocilia are abnormally short in the utricles at E15 and E16
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N |
• no pathological alterations are seen in the brain
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• kinocilia are abnormally short in the utricles at E15 and E16
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• most outer hair cells lack stereocilia at P6
• organ of Corti cultures from P1 mice show degeneration of outer hair cell stereocilia
• organ of Corti cultures from P1 mice treated with the HDAC inhibitors FK228, SAHA, or Merck60 show prevention of outer hair cell stereocilia degeneration
• mice injected with the HDAC inhibitor SAHA at P2, P4, and P5 show retention of stereocilia in outer hair cells
• SAHA treatment is more effective at rescuing the outer hair cells at P6 than at P16
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• hair cells are present until E16-17 but degenerate throughout the inner ear by P12
• the hearing organ of P6 mice shows increased apoptosis in hair cells
• the caspase inhibitors Z-DEVD-FMK and Z-VAD-FMK delay the death of hair cells in organ of Corti cultures but do not prevent the degeneration of stereocilia
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
N |
• mice are viable and survive to adulthood
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• ~60% of mice exhibit a white belly spot of variable size ranging from a few white hairs to a white spot that is ~1 cm in diameter; in contrast, only ~15% of control mice carrying the transgene alone form an extremely small, striated white-haired area on the belly
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• ~60% of mice exhibit a white belly spot of variable size ranging from a few white hairs to a white spot that is ~1 cm in diameter; in contrast, only ~15% of control mice carrying the transgene alone form an extremely small, striated white-haired area on the belly
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N |
• at E16.5, E18.5 and P0, no significant differences are observed in the number or distribution pattern of melanoblasts in the epidermis of vibrissae, back or abdominal skin relative to control mice
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• adult mice show a marked reduction in the number of Dct-LacZ+ melanoblasts and/or melanocytes in the white spot of abdominal skin
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• adult mice show a marked reduction in the number of Dct-LacZ+ melanoblasts and/or melanocytes in the white spot of abdominal skin
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
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• mice never form white spots including on the belly region
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
N |
• mice never form white spots including on the belly region
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• tamoxifen-injected mice show partial loss of hair cells
• organ of Corti cultures from P5 mice incubated with tamoxifen and treated with the HDAC inhibitors FK228 or SAHA show prevention of the tamoxifen-dependent degeneration of inner hair cells and outer hair cells
• treatment of P7-P9 tamoxifen-injected mice with SAHA fromP7 to P15 reduces the extent of tamoxifen-dependent hair cell loss in cochleas
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• in P70-P80 mice treated with tamoxifen from P40 to P47
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• mice treated with tamoxifen from P40 to P47 show hearing loss one month after treatment
• mice treated with tamoxifen from P7 to P9 show hearing loss at P16
• treatment of P7-P9 tamoxifen-injected mice with SAHA from P7 to P15 reduces the tamoxifen-dependent shift in hearing threshold at low sound frequencies
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• tamoxifen-injected mice show partial loss of hair cells
• organ of Corti cultures from P5 mice incubated with tamoxifen and treated with the HDAC inhibitors FK228 or SAHA show prevention of the tamoxifen-dependent degeneration of inner hair cells and outer hair cells
• treatment of P7-P9 tamoxifen-injected mice with SAHA fromP7 to P15 reduces the extent of tamoxifen-dependent hair cell loss in cochleas
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
N |
• mice never form white spots including on the belly region
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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 03/25/2025 MGI 6.24 |
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