All Phenotypes Tg(Nes-cre/ERT2,-ALPP)1Sbk MGI Mouse

Phenotypes associated with this allele

Jump to	Allelic Composition	Genetic Background	Genotype ID
cn1	Pten^tm2Mak/Pten^tm2Mak Tg(Nes-cre/ERT2,-ALPP)1Sbk/0	involves: 129P2/OlaHsd	MGI:5825461
cn2	Trp53^tm1Brn/Trp53^tm1Brn Tg(Nes-cre/ERT2,-ALPP)1Sbk/0	involves: 129P2/OlaHsd	MGI:5825464
cn3	Pten^tm2Mak/Pten^tm2Mak Trp53^tm1Brn/Trp53^tm1Brn Tg(Nes-cre/ERT2,-ALPP)1Sbk/0	involves: 129P2/OlaHsd	MGI:5825466

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

mortality/aging

premature death ( J:237990 )

• mice injected with tamoxifen at P0 and P1 have a median survival of 153 days

growth/size/body

megacephaly ( J:237990 )

• mice injected with tamoxifen at P0 and P1 begin to exhibit increasing macrocephaly at 2-3 months of age

behavior/neurological

lethargy ( J:237990 )

• mice injected with tamoxifen at P0 and P1 begin to exhibit lethargy at 2-3 months of age

ataxia ( J:237990 )

• mice injected with tamoxifen at P0 and P1 exhibit ataxia beginning at 2-3 months of age

seizures ( J:237990 )

• mice injected with tamoxifen at P0 and P1 exhibit seizures beginning at 2-3 months of age

nervous system

hydrocephaly ( J:237990 )

• brains of mice treated with tamoxifen at birth show hydrocephalus

increased brain size ( J:237990 )

• brains of mice treated with tamoxifen at birth show dramatic increase in overall size, especially the cerebellum

abnormal cerebellum morphology ( J:237990 )

• organization of cell layers of the cerebellum are disrupted in mice treated with tamoxifen at birth

• cerebellum hypertrophy in mice treated with tamoxifen at birth

• cerebellum shows multiple perivascular proliferative niches in mice treated with tamoxifen at birth, composed of hyperchromatic cells with progenitor-like morphology

• the perivascular hyperplastic lesions vary in size and are seen as early as 3 months of age, they grow surrounding CD34+ blood vessels and have a high percentage of Ki67+ cells

• lesions occur more frequently in anterior and lateral cerebellar lobules and in areas around the pia between lobules

abnormal cerebellar Purkinje cell layer ( J:237990 )

• Purkinje cells are not well aligned at the interface of the internal granule layer and molecular layer in mice treated with tamoxifen at birth

abnormal cerebellar granule cell morphology ( J:237990 )

• size of granule cells is larger in aging mice that were treated with tamoxifen at birth

ectopic cerebellar granule cells ( J:237990 )

• molecular layer is thickened with ectopic granule cells in mice treated with tamoxifen at birth

thin cerebellar granule layer ( J:237990 )

• the internal granule layer is thinner in mice treated with tamoxifen at birth

abnormal cerebellar molecular layer ( J:237990 )

• molecular layer is thickened with ectopic granule cells in mice treated with tamoxifen at birth

abnormal nervous system physiology ( J:237990 )

• mice injected with tamoxifen at P0 and P1 begin to exhibit progressive neurologic abnormalities at 2-3 months of age

seizures ( J:237990 )

• mice injected with tamoxifen at P0 and P1 exhibit seizures beginning at 2-3 months of age

neoplasm

neoplasm ( J:237990 )

N	• perivascular hyperplastic lesions in the cerebellum persist but do not develop into tumors up to 10 months of age

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Cowden syndrome		DOID:6457	OMIM:PS158350	J:237990

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

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Nes-cre/ERT2,-ALPP)1Sbk mutation (0 available)
Trp53^tm1Brn mutation (18 available); any Trp53 mutation (240 available)

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

neoplasm

increased tumor incidence ( J:237990 )

• 53% of mice induced with tamoxifen at P0 and P1 develop non-CNS tumors or other type of brain tumors

increased brain tumor incidence ( J:237990 )

• 53% of mice induced with tamoxifen at P0 and P1 develop non-CNS tumors or other type of brain tumors

increased medulloblastoma incidence ( J:237990 )

• 47% of mice induced with tamoxifen at P0 and P1 develop medulloblastoma with a median onset of morbidity of 135 days

nervous system

increased brain tumor incidence ( J:237990 )

• 53% of mice induced with tamoxifen at P0 and P1 develop non-CNS tumors or other type of brain tumors

increased medulloblastoma incidence ( J:237990 )

• 47% of mice induced with tamoxifen at P0 and P1 develop medulloblastoma with a median onset of morbidity of 135 days

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

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pten^tm2Mak mutation (4 available); any Pten mutation (88 available)
Tg(Nes-cre/ERT2,-ALPP)1Sbk mutation (0 available)
Trp53^tm1Brn mutation (18 available); any Trp53 mutation (240 available)

♀	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

• 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

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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