Mouse Genome Informatics
cn
    Rb1tm3Tyj/Rb1tm3Tyj
Rbl1tm1Tyj/Rbl1tm1Tyj
Tg(Pax6-cre,GFP)2Pgr/0

involves: 129S2/SvPas * 129X1/SvJ * C57BL/6 * FVB/N
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
tumorigenesis
• in 61% of mice visible unilateral retinoblastoma develop with delayed and variable kinetics appearing on average by 208 +/- 17 days relative to mice lacking Rbl2
• unlike mice lacking Rbl2, bilateral visible retinoblastomas are rare
• however, 3 of 11 retinas from mice with unilateral tumors in the opposite eye contain early retinoblastomas with mitotic figures and Homer-Wright rosettes
• at P60 4 of 14 eyes have retinoblastomas at level of the optic nerve head in the extreme periphery of the retina

vision/eye
• in 11 of 11 mice with unilateral tumors, the retina in the tumor free eye is disorganized and degenerated
• at P31 6 of 24 retinas have dysplastic lesions containing Homer-Wright rosettes at the level of the optic nerve in the extreme periphery and increased proliferation in the periphery without histological evidence of retinoblastoma

Mouse Models of Human Disease
OMIM IDRef(s)
Retinoblastoma; RB1 180200 J:119919