mortality/aging
• mice are born at the expected Mendelian ratio, but most die within 2 days
• about 25% of the homozygous mutant mice survive beyond postnatal day 2 and about 12% survive to adulthood
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behavior/neurological
• mice that escape the perinatal lethality are extremely hyperactive
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nervous system
• synapse formation was severely impaired in long-term low-density hippocampal neuron cultures derived from E18.5 brain
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• a significant reduction in activity-dependent dendritic outgrowth and significantly shorter and less complex dendritic trees than controls in long-term low-density hippocampal neuron cultures derived from embryonic brain and in brain slices
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• reduction in size of the myelinated axon bundles in P18 hippocampal cross section
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