Analysis Tools
mortality/aging
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• most mice die within 3 months with some surviving for 5 to 6 months
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nervous system
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• by 1 month, 7 of 10 mice exhibit seizures with one death during status epilepticus
• some mice exhibit hand-washing motion at the end of a seizure
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• mice exhibit abnormal activities during ictal electroencephalogram with positive spikes in the hippocampus and negative spikes in the frontal cortex and striatum followed by diffuse spike bursts
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• 88% of Arx+ GABAergic cells that migrate in wild-type mice reach the cortical plate by P0
• at P0, tangential migration of SST+ GABAergic neurons is suppressed compared to in wild-type mice
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• severe in the cortex and striatum
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• at 1 month, cholinergic neurons in the striatum, medial septum, and ventral forebrain nuclei are reduced compared to in wild-type mice
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behavior/neurological
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• compared to wild-type mice that is worse than in Arxtm3Kki homozygotes
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• mice exhibit impaired spatial learning in a win-shift task on the 8-arm radial maze compared with wild-type mice that is worse than in Arxtm3Kki homozygotes
• in a win-stay task, mice exhibit higher unlit-to-lit ratio throughout training compared with wild-type mice
• abnormal spatial learning is independent of hyperactivity and anxiety-like behaviors
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• by 1 month, 7 of 10 mice exhibit seizures with one death during status epilepticus
• some mice exhibit hand-washing motion at the end of a seizure
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• mice exhibit abnormal activities during ictal electroencephalogram with positive spikes in the hippocampus and negative spikes in the frontal cortex and striatum followed by diffuse spike bursts
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cellular
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• 88% of Arx+ GABAergic cells that migrate in wild-type mice reach the cortical plate by P0
• at P0, tangential migration of SST+ GABAergic neurons is suppressed compared to in wild-type mice
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| early infantile epileptic encephalopathy | DOID:0050709 | J:197588 | ||
