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Phenotypes Associated with This Genotype
Genotype
MGI:5009467
Allelic
Composition
Tg(TagRb)1Plm/?
Genetic
Background
involves: BALB/cJ * C57BL/6J
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phenotype observed in females
phenotype observed in males
N normal phenotype
neoplasm
• retinal tumors are first observed at 2 months of age
• tumors fill the vitreous cavity by 5-6 months of age
• tumors arise bilaterally with several foci
• tumor cells have a large hyperchromatic nucleus with minimum cytoplasm
• tumor cells have a triple nuclear membrane structure with central dense layer of either granular or fibrillar chromatin bounded on both sides by membrane
• tumor cells contain neurosecretory granules, cytoplasmic microtubules and cilia with a 9+0 pattern

vision/eye
• retinal tumors are first observed at 2 months of age
• tumors fill the vitreous cavity by 5-6 months of age
• tumors arise bilaterally with several foci
• tumor cells have a large hyperchromatic nucleus with minimum cytoplasm
• tumor cells have a triple nuclear membrane structure with central dense layer of either granular or fibrillar chromatin bounded on both sides by membrane
• tumor cells contain neurosecretory granules, cytoplasmic microtubules and cilia with a 9+0 pattern
• perivascular cuffs of tumor cells surround retinal vessels with areas of necrosis and calcification
• tumors extend by vitreous seeding and local invasion from the inner retina (endophytic) and toward the choroid (exophytic)
• tumor cells undergo photoreceptor cell differentiation to form Flexner-Wintersteiner rosettes and Homer Wright rosettes characteristic of human retinoblastoma

nervous system
• tumor cells undergo photoreceptor cell differentiation to form Flexner-Wintersteiner rosettes and Homer Wright rosettes characteristic of human retinoblastoma

Mouse Models of Human Disease
OMIM ID Ref(s)
Retinoblastoma; RB1 180200 J:10320


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
11/29/2016
MGI 6.06
The Jackson Laboratory