mortality/aging
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• die between P12-P20
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behavior/neurological
nervous system
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• fewer, shorter cells that appear disorganized
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• thinner with fewer granule cell precursors at P5 and becoming progressively worse with age
• at P8 only a single layer of granule cell precursors is present in severely affected mice
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• appears disorganized with fewer hippocampal neurons
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• fewer neurons present in the dentate gyrus at P0
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• fewer hippocampal neurons at P0 especially in the dentate gyrus
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• reduced numbers and organization of granule neurons at P7
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• at P7
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• at P7
• increase in pycnotic nuclei in the outer layer
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• reduced numbers and organization of granule neurons at P7
• decreased cholesterol levels and increased levels of sterol intermediates in granule cell precursors at P3, P5, and P7
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• at P7
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• at P7
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• cultured granule cell precursors produce fewer and thinner neurite extensions
• cell morphology is improved with the addition of cholesterol to the culture medium
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• cerebellar Purkinje cells fail to form extensive dendritic trees unlike wild-type controls
• dendritic trees eventually degenerate
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• progressively reduced proliferation of granule cell precursors in cerebella starting at P5
• increase in apoptosis of granule cell precursors in cerebella beginning at P7
• cultured granule cell precurors show reduced proliferation that is largely restored with the addition of cholesterol to the culture medium
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cellular
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• fewer, shorter cells that appear disorganized
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