Phenotypes Associated with This Genotype

Genotype
MGI:5825466

• Allelic Composition: Pten^tm2Mak/Pten^tm2Mak; Trp53^tm1Brn/Trp53^tm1Brn; Tg(Nes-cre/ERT2,-ALPP)1Sbk/0
• Genetic Background: involves: 129P2/OlaHsd

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

neoplasm

increased medulloblastoma incidence ( J:237990 )

• 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

increased glioma incidence ( J:237990 )

• 18 of 26 mice treated with tamoxifen at P0 and P1 develop gliomas in the forebrain and brain stem

nervous system

increased medulloblastoma incidence ( J:237990 )

• 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

increased glioma incidence ( J:237990 )

• 18 of 26 mice treated with tamoxifen at P0 and P1 develop gliomas in the forebrain and brain stem

abnormal cerebellum morphology ( J:237990 )

• 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