Analysis Tools
growth/size/body
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• weigh about 30% less than wild-type
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nervous system
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• exhibit impaired radial migration of neurons, resulting in abnormal hippocampal and cortical architecture
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• neuronal disorganization in Ammon's horn affecting both CA1 and CA3 regions at E14.5
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• the dentate gyrus appears chaotic at E14.5 but shows no abnormalities in the adult, suggesting a delay in its development
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• disorganization of the mossy fiber tract extending from the dentate gyrus to CA3
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• exhibit abnormal lamination of pyramidal cells in the hippocampus
• hippocampal disorganization with an additional layer of pyramidal cells in the stratum oriens that extend throughout the pyramidal cell subfields into the subiculum
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• fewer calbindin-positive pyramidal neurons in the CA1 region
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• exhibit wave-like perturbations in layer IV of the cortex (in visual, auditory, and somatosensory cortices but not in the motor or retrosplenial cortices) and to a lesser extent, in layers II/III
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• exhibit wave-like perturbations in the somatosensory cortex
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• exhibit wave-like perturbations in the visual cortex
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• exhibit wave-like perturbations in the auditory cortex
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behavior/neurological
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• exhibit impaired spatial working memory, performing worse in spontaneous alternation in a T-maze working memory task
• however, mutants perform normally in a hippocampal-independent reference memory acquisition task
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• in an elevated plus maze, heterozygotes prefer the open arms of the elevated plus and in an open field, show a reduced latency to enter the center of the open field, indicating less fear and anxiety
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• heterozygotes fail to construct proper nests
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cellular
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• exhibit impaired radial migration of neurons, resulting in abnormal hippocampal and cortical architecture
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| lissencephaly | DOID:0050453 |
OMIM:PS607432 |
J:117881 | |
