nervous system
• mutants exhibit slowly progressing photoreceptor degeneration (cone-rod dystrophy) that begins to be seen at around 9 months
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vision/eye
• mutants exhibit slowly progressing photoreceptor degeneration (cone-rod dystrophy) that begins to be seen at around 9 months
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• thinning of the outer nuclear layer starts to be seen by 9 months of age and progresses to a 20% reduction by 1 year of age
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• reduction in scotopic (a-wave) ERG amplitude starts to be seen by 9 months of age and is significantly attenuated by 12 months of age
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• reduction in photopic (b-wave) ERG amplitude starts to be seen by 9 months of age and is significantly attenuated (40% reduction) by 12 months of age
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• optomotry indicates a 15% reduction in visual acuity by 1 year of age
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