Phenotypes associated with this allele
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Myo7aHdb mutation
(1 available);
any
Myo7a mutation
(118 available)
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Stereocilia abnormalities in the Organ of Corti of Myo7aHdb/Myo7a+ and Myo7aHdb/Myo7aHdb mice at P20 and 3 months
behavior/neurological
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• increased head bobbing observed following weaning compared to heterozygotes
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• increased hyperactivity after weaning compared to heterozygotes
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hearing/vestibular/ear
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• by 3 months, very few inner hair cell stereocilia remained in the apex
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• at P20, apical inner hair cell stereocilia fusion had progressed such that the phenotype resembled the 3-month heterozygote phenotype
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• by 3 months, very few outer hair cell stereocilia remained in the apex
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• 20-23 day old mutants exhibited increased compound action potential from the round window of the cochlea across all frequencies (3-30kHz)
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nervous system
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• by 3 months, very few inner hair cell stereocilia remained in the apex
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• at P20, apical inner hair cell stereocilia fusion had progressed such that the phenotype resembled the 3-month heterozygote phenotype
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• by 3 months, very few outer hair cell stereocilia remained in the apex
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• 20-23 day old mutants exhibited increased compound action potential from the round window of the cochlea across all frequencies (3-30kHz)
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|
|
| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Myo7aHdb mutation
(1 available);
any
Myo7a mutation
(118 available)
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|
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Stereocilia defects in Myo7aHdb/Myo7a+ mice
behavior/neurological
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• head bobbing observed following weaning
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• appeared hyperactive after weaning
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hearing/vestibular/ear
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• at E18.5 and P1, apical stereocilia bundles of inner hair cells did not exhibit a height gradient and inner hair cell stereocilia were disorganized
• at P1, the basal inner hair cell stereocilia were disorganized
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• at P20, the inner hair cell stereocilia had more stereocilia sprouting from each cell that had elongated and fused together to form giant stereocilia
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• at E18.5 and P1, apical stereocilia bundles of outer hair cells did not exhibit a height gradient
• at P20, the apical outer hair cell bundles formed a more circular pattern
• at P1, basal outer hair cell stereocilia bundles formed a U-shape rather than a V-shape
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• at P20, utricles displayed bundles of very long and thin rigid stereocilia without the normal staircase arrangement while at 5 months, stereocilia bundles lost their rigidity, were splayed and lay against the utricular surface and showed wispy ends
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• 20-23 day old mutants displayed a large increase in the compound action potential thresholds from the round window of the cochlea at low frequencies (3-12 kHz)
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nervous system
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• at E18.5 and P1, apical stereocilia bundles of inner hair cells did not exhibit a height gradient and inner hair cell stereocilia were disorganized
• at P1, the basal inner hair cell stereocilia were disorganized
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|
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• at P20, the inner hair cell stereocilia had more stereocilia sprouting from each cell that had elongated and fused together to form giant stereocilia
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• at E18.5 and P1, apical stereocilia bundles of outer hair cells did not exhibit a height gradient
• at P20, the apical outer hair cell bundles formed a more circular pattern
• at P1, basal outer hair cell stereocilia bundles formed a U-shape rather than a V-shape
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• at P20, utricles displayed bundles of very long and thin rigid stereocilia without the normal staircase arrangement while at 5 months, stereocilia bundles lost their rigidity, were splayed and lay against the utricular surface and showed wispy ends
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• 20-23 day old mutants displayed a large increase in the compound action potential thresholds from the round window of the cochlea at low frequencies (3-12 kHz)
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Allelic
Composition |
Myo7aHdb/Myo7a4626SB
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Genetic
Background |
involves: BALB/c * 47BS/Rl * C3HeB/FeJ * CBA/Ca |
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Cochlear hair cell abnormalities in Myo7aHdb/Myo7a+, Myo7aHdb/Myo7a+ Myo7a4626SB/Myo7a+, and Myo7ash1/Myo7ash1 mice
hearing/vestibular/ear
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• apical stereocilia bundles of inner hair cells appeared more disorganized than in Hdb heterozygotes but less than in Hdb homozygotes
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• inner hair cell fusion was significantly reduced compared to that seen in Hdb homozygotes but not when compared to Hdb heterozygotes
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• apical stereocilia bundles of outer hair cells appeared more disorganized than in Hdb heterozygotes but less than in Hdb homozygotes
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nervous system
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• apical stereocilia bundles of inner hair cells appeared more disorganized than in Hdb heterozygotes but less than in Hdb homozygotes
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• inner hair cell fusion was significantly reduced compared to that seen in Hdb homozygotes but not when compared to Hdb heterozygotes
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• apical stereocilia bundles of outer hair cells appeared more disorganized than in Hdb heterozygotes but less than in Hdb homozygotes
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Analysis Tools