Analysis Tools
mortality/aging
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• if double transgenic pups receive doxycycline through gestation and postnatally, or through gestation but treatment stops at birth, animals appear healthy with no histologic lesions
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• if pregnant mice do not receive doxycycline (dox) during gestation, >80% of pups die in the neonatal period; double transgenic animals are never recovered
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• all ataxic mice (when dox treatment is stopped at E16) die at 3-4 weeks from a progressive neurological syndrome
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growth/size/body
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• if double transgenic pups receive doxycycline until E16, about 80% start to show growth retardation in the second postnatal week
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neoplasm
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• necropsy of animals dying at 3-4 weeks shows infiltrative lesions of cerebellar hemispheres composed of tumor cells (mitotically active, primitive-appearing cells with hyperchromatic nuclei and little cytoplasm)
• when affected mice start to receive dox again at P16, tumors show less necrosis and apoptosis than untreated mutants
• pups which received doxycline to birth with cessation at P0 have normal cerebellar architecture and do not show tumor formation or migration abnormalities of granule cell precursors
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behavior/neurological
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• if double transgenic pups receive doxycycline until E16, about 80% develop tremor around P12
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• if double transgenic pups receive doxycycline until E16, about 80% develop ataxia around P12
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nervous system
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• necropsy of animals dying at 3-4 weeks shows infiltrative lesions of cerebellar hemispheres composed of tumor cells (mitotically active, primitive-appearing cells with hyperchromatic nuclei and little cytoplasm)
• when affected mice start to receive dox again at P16, tumors show less necrosis and apoptosis than untreated mutants
• pups which received doxycline to birth with cessation at P0 have normal cerebellar architecture and do not show tumor formation or migration abnormalities of granule cell precursors
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• tumor cells are observed primarily in the molecular layer and external granule layer, but also frequently invading the deep cerebellar white matter and brainstem structures
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• at P12, mice which received doxycycline until E16 display lesions confined to the EGL which shows a diffuse hyperplasia throughout the cerebellar hemispheres
• at P16, affected animals show marked proliferation of the EGL with diffuse infiltration of the molecular layer, consistent with malignant tumor formation; in contrast, control animals exhibit complete regression of the EGL
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| medulloblastoma | DOID:0050902 |
OMIM:155255 |
J:94092 | |
