Analysis Tools
vision/eye
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• abnormalities are more evident as expression levels of the transgene increase in the three lines created
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• mice exhibit localized regions of the retina that appear lighter because of depigmentation in areas of retinal pigment epithelium atrophy and photoreceptor degeneration
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• as early as 3 weeks, the photoreceptor outer segment is severely stunted with disks clustered in groups instead of stacked as in wild-type mice
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• at 6 weeks, 50% of photoreceptor in the outer nuclear layer are degenerated
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• more severe in the center of the retina
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• retinal degeneration is progressive, localized, and more severe in the center of the retina
• retinal degeneration is more severe than in other lines over expressing the same transgene
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• at rod-saturation, cone b-wave declines in amplitude compared to in wild-type mice
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• rod b-wave response is reduced at 22 weeks and absent at 22 weeks
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nervous system
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• as early as 3 weeks, the photoreceptor outer segment is severely stunted with disks clustered in groups instead of stacked as in wild-type mice
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• at 6 weeks, 50% of photoreceptor in the outer nuclear layer are degenerated
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pigmentation
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• more severe in the center of the retina
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Stargardt disease | DOID:0050817 |
OMIM:248200 OMIM:600110 OMIM:603786 |
J:97173 | |
