behavior/neurological
• homozygotes are unable to stand and walk across a narrow bar and instead pull themselves across with their forepaws
• homozygotes fall off a rotating rod more quickly than wild-type
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nervous system
• cerebellar development is delayed with hypoplasia of the folia and fissure and retarded layer formation until 2 weeks of age
• at 9 weeks of age, the intercrucial fissure is almost absent; however, other structures are mostly normal
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• at P5 and P7 hypoplasia of cerebellar folia is seen
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• significant delay in granule cell migration during the first 2 weeks of life, but no change in granule cell proliferation
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• during the first 2 postnatal weeks, Bergmann glia extend fewer radial fibers, but this becomes less pronounced by 3 weeks of age
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• during the first 2 postnatal weeks, Bergmann glia are found in the molecular layer, but this becomes less pronounced by 3 weeks of age
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• many Purkinje cells are innervated with multiple climbing fibers
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• with high intensity stimulation 41% of excitatory postsynaptic currents show 2 to 3 discrete steps compared to only 11% of wild-type mice with excitatory postsynaptic currents showing 2 discrete
• high-frequency stimulation evoked excitatory postsynaptic currents show decreased amplitude and pronounced temporal summation suggesting reduced glutamate uptake
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• high-frequency stimulation evoked excitatory postsynaptic currents show decreased amplitude
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