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Phenotypes Associated with This Genotype
Genotype
MGI:3831004
Allelic
Composition
Tg(Neurod2-Smo*A1)199Jols/Tg(Neurod2-Smo*A1)199Jols
Genetic
Background
C57BL/6-Tg(Neurod2-Smo*A1)199Jols
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Neurod2-Smo*A1)199Jols mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mice displaying clinical symptoms of tumor formation such as a protruding skull, head tilt, hunched posture (all suggesting hydrocephalus), ataxia (suggesting cerebellar abnormalities) or weight loss are sacrificed

growth/size/body

nervous system
N
• mice exhibit normal cerebellum development
• by 5 months, incidence is >80% (J:133312)
• tumors contain polygonal to elongate cells in densely cellular sheets with rosette formation, celluar palisading, and frequent mitoses (J:133312)
• large tumors show areas of necrosis and neovascularization (J:133312)
• tumor invasion into fourth ventricle is seen in some animals (J:133312)
• tumors contain highly proliferative progenitor-like cells and are characterized by significant loss of neuronal differentiation (J:133312)
• asymptomatic animals examined histologically at 1 and 2 months had tumor incidences of 84 and 95% respectively, with tumors localized mainly to the surface of the cerebellum (J:133312)
• medulloblastomas with cerebellar dysplasia and subsequent cerebellar effacement are observed in majority of affected mice
• in addition to tumors seen at 1 and 2 months, animals also show ectopic granule cell rests in the superficial and midmolecular layers (in 63% of affected mice at 1 month and 68% at 2 months); such rests contain differentiated cells in contrast to cells in the cancer foci
• transplantation of tumors to wild-type recipients result in cerebellar tumors
• at P14, all mice show thickening of external granule layer (EGL) with no evidence of tumor formation at this time; thickness is 2-4 cells thick in wild-type but is 12-20 cells in transgenic cerebella
• thickness is comparable at P5
• mice display leptomeningeal spreading tumors on brain surface and within spinal cord with foci of neoplastic cells detected in the brain distant from original tumor with vasculature often present at these sites

behavior/neurological

craniofacial

skeleton

neoplasm
• by 5 months, incidence is >80% (J:133312)
• tumors contain polygonal to elongate cells in densely cellular sheets with rosette formation, celluar palisading, and frequent mitoses (J:133312)
• large tumors show areas of necrosis and neovascularization (J:133312)
• tumor invasion into fourth ventricle is seen in some animals (J:133312)
• tumors contain highly proliferative progenitor-like cells and are characterized by significant loss of neuronal differentiation (J:133312)
• asymptomatic animals examined histologically at 1 and 2 months had tumor incidences of 84 and 95% respectively, with tumors localized mainly to the surface of the cerebellum (J:133312)

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
medulloblastoma DOID:0050902 OMIM:155255
J:189258


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