vision/eye
• the outer segment of cells in the outer nuclear layer are disrupted in number and configuration compared to in wild-type mice
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• whorl-like structures
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• fewer phagosomes are observed than in wild-type mice despite ongoing phagocytosis of the membrane
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• at 6 months, the outer nuclear layer (ONL) is only 4 or 5 nuclei thick and there is evidence of ongoing depletion of cells unlike in wild-type mice
• at 10 months, the ONL is only 3 or 4 nuclei thick unlike in wild-type mice
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• mice exhibit 9 or 10 rows of photoreceptor nuclei at 2 months, 4 or 5 rows at 6 months, and 2 or 3 rows at 12 months of age indicating a faster degeneration than in Prph2Rd2 heterozygotes
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• at 6 months, a- and b-wave of dark-adapted eyes are less than in wild-type mice
• at 10 months, a- and b-waves, maximal dark-adapted responses, and cone-isolated responses are further reduced
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nervous system
• the outer segment of cells in the outer nuclear layer are disrupted in number and configuration compared to in wild-type mice
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• whorl-like structures
|
pigmentation
• fewer phagosomes are observed than in wild-type mice despite ongoing phagocytosis of the membrane
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
retinitis pigmentosa 7 | DOID:0110383 |
OMIM:608133 |
J:76490 |