vision/eye
• TUNEL assays showed increased retina apoptosis at 15 months of age; TUNEL+ cells are mostly found in the outer nuclear layer (ONL), indicating continuous photoreceptor cell death
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• inner nuclear layer (INL) thickness is normal at 9 months but slightly decreased at 15 months of age
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• outer nuclear layer (ONL) thickness is progressively reduced starting at 9 months of age and ONL is drastically thinner by 15 months age
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• thickness of the photoreceptor layer (inner segment + outer segment) is progressively decreased starting at 9 months of age and drastically reduced by 15 months age
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• number of cone cells is decreased at 12 months and drastically reduced by 15 months of age
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• photoreceptor inner segment is significantly shortened at 15 months of age, as shown by decreased Na-K ATPase staining
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• photoreceptor outer segment is significantly shortened at 12 and 15 months of age, as shown by decreased rhodopsin staining
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• mice show late-onset degeneration of photoreceptor cells
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• progressive degeneration of cone cells
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• progressive degeneration of rod cells
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• mean a-wave amplitudes are reduced by ~15% in scotopic response tests
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• mean b-wave amplitudes are reduced by ~30% in scotopic response tests
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• all mice show attenuated electroretinogram (ERG) responses around 8 months of age
• however, ERG responses are normal at 6 months of age
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• all mice exhibit reduced photopic responses at 8 months of age with a somewhat flat wave under light-adapted conditions
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• all mice exhibit reduced scotopic responses at 8 months of age
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nervous system
• number of cone cells is decreased at 12 months and drastically reduced by 15 months of age
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• photoreceptor inner segment is significantly shortened at 15 months of age, as shown by decreased Na-K ATPase staining
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• photoreceptor outer segment is significantly shortened at 12 and 15 months of age, as shown by decreased rhodopsin staining
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• mice show late-onset degeneration of photoreceptor cells
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• progressive degeneration of cone cells
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• progressive degeneration of rod cells
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cellular
• TUNEL assays showed increased retina apoptosis at 15 months of age; TUNEL+ cells are mostly found in the outer nuclear layer (ONL), indicating continuous photoreceptor cell death
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