Analysis Tools
nervous system
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• at both early and late stages of corticogenesis, many actively dividing cells are abnormally positioned in the brain which is associated with increased cell death in cortices at E13
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• fewer radial glial cells in mitosis are apparent at the ventricular lining
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• misplaced progenitor cells in E13 brains, present outside the ventricular zones, continue to proliferate longer than wild-type cells
• total number of BrdU+ cells is greater and more cells remain in the cell cycle, particularly in the subventricular zone, intermediate zone and cortical plate
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• mice exhibit early corticogenesis progenitor defects in the brain
• at P3, many Cux1+ neurons fail to reach cortical layers II-IV and are present in the heterotopia, however almost all Tbr1+ neurons have reached their final destination in layer VI above the heterotopia
• at P3, and to a lesser extent at P7, columns of Cux1+ neurons between the heterotopia and the cortex are still seen, whereas the migration of these neurons is already complete in wild-type mice, indicating a temporal delay in neurons reaching their destination
• however, neurons migrate at the same speed as wild-type neurons
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• ectopic proliferation in the intermediate zone during corticogenesis
• many highly proliferating neuronal cells born at E15.5 remain blocked in the lower intermediate zone
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• mice present subcortical heterotopia in the rostromedial part of the neocortex close to the hippocampus
• by E17, both early-born, Tbr1+ and late-born, Cux1+ neurons (apical progenitors) contribute to heterotopia formation and disorganized radial glial cell processes are seen throughout the heteropia
• however, accumulation of heterotopic neurons is not seen at E15
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cellular
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• most anaphase cells located at the ventricular lining show different cleavage orientations at E13 and E16 from the wild-type cells which show vertically oriented DNA, indicating abnormal spindle orientations
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• at both early and late stages of corticogenesis, many actively dividing cells are abnormally positioned in the brain which is associated with increased cell death in cortices at E13
|
|
• fewer radial glial cells in mitosis are apparent at the ventricular lining
|
|
• misplaced progenitor cells in E13 brains, present outside the ventricular zones, continue to proliferate longer than wild-type cells
• total number of BrdU+ cells is greater and more cells remain in the cell cycle, particularly in the subventricular zone, intermediate zone and cortical plate
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| congenital nervous system abnormality | DOID:2490 | J:211342 | ||
