behavior/neurological
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• subtle but abnormal motion of the body swaying laterally is seen at about 21 days of age
• persistent mild to moderate ataxia is noticeable throughout life, however it does not worsen with age
• width of steps is narrower, but do not see widening of the distance between both feet
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• cannot stay on the rotarod at all and fall down within the first 2-3 seconds
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nervous system
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• greater density of stellate neurons along with reactive glia
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• low in 4.5 month old mice (389 mg vs. 503 mg in controls)
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• incomplete foliation, particularly in the central lobules
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• 50% loss of Purkinje cells at 3 weeks of age, with cell loss rapidly accelerating so that by 6 weeks of age, almost all are lost, however do not see any severe loss of neural cells in the cerebellum and brainstem
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• thinning of the granular layer seems to be due to a decrease in the number of granule cells per unit area
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• thinner at 4.5 months of age but not at 3 weeks of age, due to loss of Purkinje dendritic trees
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• small in 4.5 month old mice
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reproductive system
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• mature sperm are seldom seen in the testis, however primary spermatocytes and spermatogonia are observed in the seminiferous tubules
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• cessation of differentiation mainly at the spermatid stage and degeneration of cells
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• although females are infertile, no pathological change in the ovaries is observed and ova can be fertilized in vitro
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cellular
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• mature sperm are seldom seen in the testis, however primary spermatocytes and spermatogonia are observed in the seminiferous tubules
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