Analysis Tools
nervous system
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• progressive degeneration of photoreceptors
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• by P70, a decrease in cone cell number is seen with 40-50% loss and nearly complete cone cell loss is seen at P125
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vision/eye
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• progressive degeneration of photoreceptors
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• by P70, a decrease in cone cell number is seen with 40-50% loss and nearly complete cone cell loss is seen at P125
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• by P70, a decrease in the outer nuclear layer thickness is seen, with a loss of 4-5 cell layers
• outer nuclear layer thickness in the peripheral retina is decreased with loss of about 43% of outer nuclei at P125, however outer nuclear layer thickness is stable in the central retina between P70 and P125
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• mice show reduced photopic, cone-mediated ERG responses with about 30-40% of ERG responses from controls at P15
• mice show a rapid decline in photopic cone-mediated ERG responses decreasing to about 10% of control levels by P60
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• mice show reduced scotopic, rod-mediated ERG responses to about 50-70% of ERG responses of controls, with greater reduction at lower flash intensities, at P15
• rods exhibit a higher half-saturating light intensity, indicating reduced sensitivity
• scotopic, rod-mediated ERG responses decrease at a much slower rate, with the largest decline seen between P60 and P90
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• at P30, mice show lower threshold spatial frequencies of the gratings that elicit an optokinetic response than controls under photopic conditions, which worsens at P60, indicating lower visual acuity
• mice sow a trend toward lower visual acuity under scotopic conditions, with significantly lower spatial frequencies under 3 of 5 scotopic background light intensities at P30 and by P60, visual behavior is lowered under all scotopic light intensities
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| cone-rod dystrophy | DOID:0050572 | J:218096 | ||
