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Phenotypes Associated with This Genotype
Genotype
MGI:5825466
Allelic
Composition
Ptentm2Mak/Ptentm2Mak
Trp53tm1Brn/Trp53tm1Brn
Tg(Nes-cre/ERT2,-ALPP)1Sbk/0
Genetic
Background
involves: 129P2/OlaHsd
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ptentm2Mak mutation (4 available); any Pten mutation (49 available)
Tg(Nes-cre/ERT2,-ALPP)1Sbk mutation (0 available)
Trp53tm1Brn mutation (17 available); any Trp53 mutation (155 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
neoplasm
• 100% of mice induced with tamoxifen at P0 and P1 develop medulloblastoma with a median onset of morbidity of 63 days of age
• medullablastomas of tamoxifen treated mice show extensive and abnormal vascularization and highly differentiated tumor cells surrounding a perivascular tumor stem cell niche that is not seen in single Trp53 conditional mutants
• blood clots are often seen in the lumen of abnormal blood vessels
• medulloblastoma initiates from a perivascular tumor stem cell niche
• medulloblastomas of tamoxifen treated mice acquire somatic inactivation of Ptch1 and show expression signatures of sonic hedgehog subgroup medulloblastoma
• 18 of 26 mice treated with tamoxifen at P0 and P1 develop gliomas in the forebrain and brain stem

nervous system
• 100% of mice induced with tamoxifen at P0 and P1 develop medulloblastoma with a median onset of morbidity of 63 days of age
• medullablastomas of tamoxifen treated mice show extensive and abnormal vascularization and highly differentiated tumor cells surrounding a perivascular tumor stem cell niche that is not seen in single Trp53 conditional mutants
• blood clots are often seen in the lumen of abnormal blood vessels
• medulloblastoma initiates from a perivascular tumor stem cell niche
• medulloblastomas of tamoxifen treated mice acquire somatic inactivation of Ptch1 and show expression signatures of sonic hedgehog subgroup medulloblastoma
• 18 of 26 mice treated with tamoxifen at P0 and P1 develop gliomas in the forebrain and brain stem
• cerebellum at P21 of mice treated with tamoxifen at birth shows disrupted lamination and abundant Ki67+ proliferative cells throughout the cerebellum , which are concentrated around blood vessels either under the pial surface or in the parenchyma

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
medulloblastoma DOID:0050902 OMIM:155255
J:237990


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB), Gene Ontology (GO)
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last database update
04/06/2021
MGI 6.16
The Jackson Laboratory