nervous system
• at P50, mice exhibit a progressive and substantial (73%) loss of Purkinje cells in the anterior and posterior lobes of the vermis
• at 5 months of age, mice exhibit an 85% loss of Purkinje cells compared to in wild-type mice
• loss of Purkinje cells is due to apoptosis
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• at P45, deep cerebellar nuclei exhibit progressive neurodegeneration
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• prior to neurodegeneration, axons of the optic nerve, brainstem, spinal cord and frontal cortex exhibit an accumulation of neurofilament-L and axonal swelling unlike in wild-type mice
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• at 4 months of age, the average number of ganglionic cells per 100 um is reduced by one-third compared to in wild-type mice
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• at 4 months of age, mice exhibit neurodegeneration of the Purkinje cells, deep cerebellar nuclei and retinal ganglion cells
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• at 4 months of age, the average number of ganglionic cells per 100 um is reduced by one-third compared to in wild-type mice
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behavior/neurological
vision/eye
• at 4 months of age, the average number of ganglionic cells per 100 um is reduced by one-third compared to in wild-type mice
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• at 4 months of age, the average number of ganglionic cells per 100 um is reduced by one-third compared to in wild-type mice
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• at 4 months of age
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• at 4 months of age
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