Analysis Tools
vision/eye
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• outer segment disc disorganization and accumulation of extracellular debris is seen at P25 and P28
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• mice show a shortening of the outer segment length at 1 month of age
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• at P31, the outer segments are disorganized and appear to lose the connection with the axoneme and innersegment of the photoreceptor cell
• mice show mislocalization of photoreceptor outer segment proteins
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• outer segments are fully degenerated by P31
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• mice show thinning of the outer nuclear layer at 1 month, with a 38% reduction, and a complete degeneration by 2 months
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• mice exhibit rapid retinal degeneration showing decreased neural retinal thickness due to thinning and eventual loss of the outer nuclear layer by 2 months of age
• however, mice do not show retinal cysts
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• mixed rod/cone responses are reduced by half at 1 month of age
• by 2 months of age, the ERG is severely affected or undetectable across the stimulus conditions
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• mice show an 84% reduction of rod-driven b-wave amplitude at 0.01 cd/m2 light stimulus after dark adaptation at 1 month of age
• mice show a reduction in cone-isolated b-wave amplitude at 20 cd/m2 light stimulus after light adaptation at 2 months of age, indicating secondary cone degeneration
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nervous system
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• outer segment disc disorganization and accumulation of extracellular debris is seen at P25 and P28
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• mice show a shortening of the outer segment length at 1 month of age
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• at P31, the outer segments are disorganized and appear to lose the connection with the axoneme and innersegment of the photoreceptor cell
• mice show mislocalization of photoreceptor outer segment proteins
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• outer segments are fully degenerated by P31
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| retinal degeneration | DOID:8466 | J:262800 | ||
