Phenotypes associated with this allele
neoplasm
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• mice injected intrasplenicaly with adenovirus expressing Cre recombinase develop multiple liver lesions after a latency of 3-4 months
• tumors resemble human hepatocellular carcinoma
• expansion of populations of stem/progenitor cells suggests that these cells, not hepatocytes, are the initiating populations in hepatocellular carcinoma development
• mutants treated with DAPT, a gamma-secretase inhibitor, 75 days after adenoviral Cre injection, show macroscopically visible tumors 12 days after the beginning of treatment
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liver/biliary system
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• mice injected intrasplenicaly with adenovirus expressing Cre recombinase develop multiple liver lesions after a latency of 3-4 months
• tumors resemble human hepatocellular carcinoma
• expansion of populations of stem/progenitor cells suggests that these cells, not hepatocytes, are the initiating populations in hepatocellular carcinoma development
• mutants treated with DAPT, a gamma-secretase inhibitor, 75 days after adenoviral Cre injection, show macroscopically visible tumors 12 days after the beginning of treatment
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Rb1tm3Tyj mutation
(10 available);
any
Rb1 mutation
(106 available)
Rbl2tm2.1Tyj mutation
(0 available);
any
Rbl2 mutation
(52 available)
Tg(Pax6-cre,GFP)2Pgr mutation
(1 available)
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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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Analysis Tools