Analysis Tools
vision/eye
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• a small number of photoreceptors exhibit spreading microtubles in the distal part of the connecting cilium
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• retinas at 43 weeks of age are depigmented and depleted of photoreceptors
• initiation of photoreceptor cell death appears at 4 weeks of age
• photoreceptor cell death occurs during outer nuclear layer (ONL) thinning at 7 weeks
• both rod and cone photoreceptors degenerate concurrently in the retina from week 4 to 10
• however, counts of retinal bipolar cells, amacrine cells, and retinal ganglion cells are not different and the outer segment and basal body appear normal
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• retinal pathology progresses from a blurred ellipsoid zone and reduced outer nuclear layer thickness at 4 weeks to more thinned ONL at 7 weeks and 10 weeks
• however, the inner nuclear layer is maintained
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• ERG responses from rod and cone photoreceptors progressively decrease from week 4 to 10 and ERG responses are not recordable at 43 weeks
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• ERG indicates reduced scotopic a-wave and photopic a-wave
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nervous system
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• a small number of photoreceptors exhibit spreading microtubles in the distal part of the connecting cilium
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• retinas at 43 weeks of age are depigmented and depleted of photoreceptors
• initiation of photoreceptor cell death appears at 4 weeks of age
• photoreceptor cell death occurs during outer nuclear layer (ONL) thinning at 7 weeks
• both rod and cone photoreceptors degenerate concurrently in the retina from week 4 to 10
• however, counts of retinal bipolar cells, amacrine cells, and retinal ganglion cells are not different and the outer segment and basal body appear normal
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cellular
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• a small number of photoreceptors exhibit spreading microtubles in the distal part of the connecting cilium
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