Mouse Genome Informatics
tg
    Tg(TagRb)1Plm/?
involves: BALB/cJ * C57BL/6J
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
tumorigenesis
• 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
• 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 IDRef(s)
Retinoblastoma; RB1 180200 J:10320