vision/eye
• at 48 days, the retinal outer segment and retinal inner segment are thinned further than at earlier time points unlike in wild-type mice
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• at 27 days
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• the retinal outer segment is thinner than in wild-type or heterozygous mice
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• at 28 days, the outer nuclear layer (ONL) is 8 to 10 nuclei thick compared to 10 to 12 nucleic in wild-type mice
• at 48 days, the ONL is reduced to half of the width at 24 days
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• degeneration is complete by 3 months
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• light-adapted mice exhibit a somewhat reduced cone response without an a-wave unlike in wild-type mice
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• dark-adapted mice exhibit no electroretinography responses below -0.3 cd/m2 and while small b-waves are generated at high intensities no a-wave is observed
• b-wave response is narrower with a faster time course than in wild-type mice and lacks most of the oscillatory potential observed on the leading edge of the dark-adapted b-waves in wild-type mice
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nervous system
• at 48 days, the retinal outer segment and retinal inner segment are thinned further than at earlier time points unlike in wild-type mice
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• at 27 days
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• the retinal outer segment is thinner than in wild-type or heterozygous mice
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
retinitis pigmentosa 4 | DOID:0110372 |
OMIM:613731 |
J:38098 |