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Phenotypes Associated with This Genotype
Genotype
MGI:3707433
Allelic
Composition
Rb1tm3Tyj/Rb1tm3Tyj
Rbl1tm1Tyj/Rbl1tm1Tyj
Tg(Pax6-cre,GFP)2Pgr/0
Genetic
Background
involves: 129S2/SvPas * 129X1/SvJ * C57BL/6 * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Rb1tm3Tyj mutation (1 available); any Rb1 mutation (34 available)
Rbl1tm1Tyj mutation (1 available); any Rbl1 mutation (12 available)
Tg(Pax6-cre,GFP)2Pgr mutation (1 available)
phenotype observed in females
phenotype observed in males
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 (J:119919)
• unlike mice lacking Rbl2, bilateral visible retinoblastomas are rare (J:119919)
• however, 3 of 11 retinas from mice with unilateral tumors in the opposite eye contain early retinoblastomas with mitotic figures and Homer-Wright rosettes (J:119919)
• at P60 4 of 14 eyes have retinoblastomas at level of the optic nerve head in the extreme periphery of the retina (J:119919)
• 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 (J:119919)
• unlike mice lacking Rbl2, bilateral visible retinoblastomas are rare (J:119919)
• however, 3 of 11 retinas from mice with unilateral tumors in the opposite eye contain early retinoblastomas with mitotic figures and Homer-Wright rosettes (J:119919)
• at P60 4 of 14 eyes have retinoblastomas at level of the optic nerve head in the extreme periphery of the retina (J:119919)

vision/eye
• in 11 of 11 mice with unilateral tumors, the retina in the tumor free eye is disorganized and degenerated (J:119919)
• 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 (J:119919)
• in 11 of 11 mice with unilateral tumors, the retina in the tumor free eye is disorganized and degenerated (J:119919)
• 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 (J:119919)

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


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
02/02/2016
MGI 6.02
The Jackson Laboratory