Analysis Tools
vision/eye
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• a gradual thinning of the photoreceptor outer layer is observed with age
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• up to 10 months of age, photoreceptor morphology is normal, but photoreceptors are under stress and are in a pre-degenerating state
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• shortening of inner/outer segments is seen with age
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• shortening of inner/outer segments is seen with age
• at 20 months of age, outer segments become very short and disorganized
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• as mice age, signs of photoreceptor degeneration are observed
• at 20 months of age, >50% of photoreceptors are lost
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• cone photoreceptors undergo degeneration by 20 months of age
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• by 20 months of age, a- and b-waves are reduced by >60% compared to wild-type controls
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nervous system
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• widespread loss of outer hair cells in the basal turn is observed
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• up to 10 months of age, photoreceptor morphology is normal, but photoreceptors are under stress and are in a pre-degenerating state
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• shortening of inner/outer segments is seen with age
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|
• shortening of inner/outer segments is seen with age
• at 20 months of age, outer segments become very short and disorganized
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• as mice age, signs of photoreceptor degeneration are observed
• at 20 months of age, >50% of photoreceptors are lost
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• cone photoreceptors undergo degeneration by 20 months of age
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hearing/vestibular/ear
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• widespread loss of outer hair cells in the basal turn is observed
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• at 4 months of age, mice show normal cochlear function at low frequency but an elevated threshold at higher frequencies; there was no progression when tested at 7 months of age
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Usher syndrome type 2A | DOID:0110838 |
OMIM:276901 |
J:118927 | |
