Analysis Tools
mortality/aging
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• mice are moribund by 183 +/- 39 days
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neoplasm
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• visible bilateral retinoblastoma develop with rapid and consistent kinetics appearing on average at 128 +/- 18 days
• amacrine cells, a subset of progenitor cells, and Muller cells are present in tumors
• at P31 (4 animals) and P60 (3 animals) large tumors are present and all mice have retinoblastomas in each eye that seed the vitreous and anterior chamber by 4 months of age
• by 183 +/- 39 days mice have grossly distended eyes where the tumor has filled the anterior chambers and often invaded of local tissues
• tumors infiltrate the optic nerve and metastases are found in the cervical lymph nodes (11 of 28) and brain (7 of 27)
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vision/eye
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• at P12, apoptosis is increased and at P21 retinas contain apoptotic bodies
• the increase in apoptosis is greater than in mutant mice wild-type for Rbl2
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• visible bilateral retinoblastoma develop with rapid and consistent kinetics appearing on average at 128 +/- 18 days
• amacrine cells, a subset of progenitor cells, and Muller cells are present in tumors
• at P31 (4 animals) and P60 (3 animals) large tumors are present and all mice have retinoblastomas in each eye that seed the vitreous and anterior chamber by 4 months of age
• by 183 +/- 39 days mice have grossly distended eyes where the tumor has filled the anterior chambers and often invaded of local tissues
• tumors infiltrate the optic nerve and metastases are found in the cervical lymph nodes (11 of 28) and brain (7 of 27)
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• at P12 in the retinal periphery proliferation and apoptosis are increased and proliferation remains elevated at P21,especially in the extreme periphery
• proliferation is increased and prolonged relative to mutant mice wild-type for Rbl2
• at P21 retinas are very hypocellular, contain apoptotic bodies and many cells with large and/or irregular-shaped nuclei, and 9 of 12 have early dysplatic lesions containing Homer-Wright rosettes in the extreme periphery
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• at P21, the amacrine layer is significantly reduced away from tumor areas
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• rod bipolar cells are very rare or absent from retinas and retinoblastomas
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• increase in horizontal cells in contrast to the general hypocellularity of the retina
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• at P21, the three nuclear layers can not be distinguished, except in the central retina where Cre expression is reduced
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• Tuji-positive retinal ganglion cells are very rare or completely absent from retinas and tumors
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• a cone subset (positive for M-opsin) is very rare or absent from retinas and retinoblastomas
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• despite the increase in proliferation retinas are very hypoplastic at P21
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nervous system
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• Tuji-positive retinal ganglion cells are very rare or completely absent from retinas and tumors
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• a cone subset (positive for M-opsin) is very rare or absent from retinas and retinoblastomas
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• at P21, the amacrine layer is significantly reduced away from tumor areas
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• rod bipolar cells are very rare or absent from retinas and retinoblastomas
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• increase in horizontal cells in contrast to the general hypocellularity of the retina
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cellular
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• at P12, apoptosis is increased and at P21 retinas contain apoptotic bodies
• the increase in apoptosis is greater than in mutant mice wild-type for Rbl2
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| retinoblastoma | DOID:768 |
OMIM:180200 |
J:119919 | |
