mortality/aging
• less than 10% of mice survive to 5 months of age
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• about half the die mice before weaning
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behavior/neurological
• repetitive epileptiform discharges from the hippocampus consisting of 4-Hz monomorphic spike activity are observed in mice prior to seizures
• at the onset of the convulsive attack, hippocampus activity increases from 400 microV up to 1 mV, and then transitions into spike and wave appearance at 3-Hz frequency
• these spikes in activity transmit to the cortex about half the time leading to tonic-clonic convulsions
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• as early as P16, mice have spontaneous seizures often accompanied by behavioral arrest and forelimb myoclonus
• seizures originate in the hippocampus with spikes in activity that half the time transmit to the cortex and cause tonic-clonic seizures
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nervous system
• repetitive epileptiform discharges from the hippocampus consisting of 4-Hz monomorphic spike activity are observed in mice prior to seizures
• at the onset of the convulsive attack, hippocampus activity increases from 400 microV up to 1 mV, and then transitions into spike and wave appearance at 3-Hz frequency
• these spikes in activity transmit to the cortex about half the time leading to tonic-clonic convulsions
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• as early as P16, mice have spontaneous seizures often accompanied by behavioral arrest and forelimb myoclonus
• seizures originate in the hippocampus with spikes in activity that half the time transmit to the cortex and cause tonic-clonic seizures
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• CaBP+ interneurons that interact with pyramidal neurons are improperly laminated within the stratum radiatum-lacunosum moleculare border
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• mice have reduced packing density of the dentate granule neuron layer, resulting in an increased thickness
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• mossy fibers fail to separate into the infrapyramidal and the suprapyrimidal bundles and, as a result, mossy fibers travel aberrantly within the pyramidal layer
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• lamination density of the granule cell layer is increased in these mice
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• pyramidal neurons displayed a striking simplification of apical dendritic arbors with decreased branching from both primary and secondary apical dendrites
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• about half of the somatostatin neurons are selectively lost in the hippocampus by day 18 after birth
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• about 40% of neurons are displaced into the stratum oriens
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• the CA3 region is dyslaminated with a small percentage of neurons heterotopically located in the stratum oriens
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• the frequency of spontaneous sIPSC among pyramidal neurons is severely reduced by 55% compared to controls
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