Analysis Tools
mortality/aging
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• animals that were small tended to die between P5 and P21
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behavior/neurological
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• mutants that survived to adulthood appeared jittery
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• mutants that survived to adulthood appeared to have normal life span but were solitary
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• mutants that survived to adulthood exhibited sporadic seizures
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growth/size/body
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• most mutant mice were slightly smaller than controls but were within the normal range of sizes and survived to adulthood
• occasionally, they reached only about one-third the size of their littermates
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nervous system
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• mutants that survived to adulthood exhibited sporadic seizures
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• TUNEL staining of E14.5 embryonic brain sections suggest that increased cell death may contribute to the loss of mature neurons
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• several experiments indicate that neural progenitor cell proliferation increased drastically in the mutant region
• starting from E10.5, Nestin-positive progenitor cells in the mutant showed very little radial organization
• mutant neural progenitor cells were organized into multiple clusters of neurogenic foci across the cortex and did not migrate to the outer layer of the cortex
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• by E10.5, the neuroepithelium in the dorsal forebrain was noticeably undulating
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• rough ventricular surfaces in mutant brains are suggestive of the shedding of brain tissues into the ventricle; shed tissues were occasionally found in the lateral ventricles
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• extensive ventricular enlargement
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• by E10.5, the neuroepithelium in the dorsal forebrain was noticeably undulating
• at E12.5, the forebrain exhibited undulating, folding, and invaginating with clumps of cells peeling into the lateral ventricle from the apical surface of the cortical ventricular zone
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• marginal zone absent and displayed no discernible laminar structures
• contained numerous cell clusters
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• shed tissues were occasionally found in the lateral ventricles
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• no identifiable corpus callosum in the caudodorsal region while corpus callosum in the rostral region was relatively normal
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• small thalamus
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• overgrown caudodorsal neocortex and thinning of the lateral cortex
• in extreme cases, the caudodorsal region was nearly missing, with just a thin layer of the cortex remaining with extreme ventricular enlargement
• the volume of the mutant cortex was increased
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• reduction in the number of mature neurons later in the double mutant animal was revealed by reduced expression of neuronal markers
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• multidendritic neurons, normally located in layer I, were distributed all over the mutant cortex
• Cajal-Retzius cells were displaced from the normal location in the outer layer of the neocortex to the ectopic sites across the cortex
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reproductive system
hearing/vestibular/ear
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• 25% of the mutants displayed hypersensitivity to sound stimuli
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embryo
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• by E10.5, the neuroepithelium in the dorsal forebrain was noticeably undulating
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cellular
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• TUNEL staining of E14.5 embryonic brain sections suggest that increased cell death may contribute to the loss of mature neurons
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• several experiments indicate that neural progenitor cell proliferation increased drastically in the mutant region
• starting from E10.5, Nestin-positive progenitor cells in the mutant showed very little radial organization
• mutant neural progenitor cells were organized into multiple clusters of neurogenic foci across the cortex and did not migrate to the outer layer of the cortex
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