behavior/neurological
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• in some mice
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• on a rotarod and balance beam
• however, treatment with acetazolamide or transgene complementation improves rotarod performance
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• flattened on a balance beam
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• beginning at P7, some mice exhibit abnormal gait with higher stance and splayed hind limbs unlike wild-type mice
• gait abnormality is more severe at P10
• some mice exhibit increased stride length and width and increased toe spread compared with wild-type mice
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nervous system
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• the frequency of Purkinje cell action potential firing is reduced compared to in wild-type mice
• however, basket cell firing rate is normal
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• Purkinje cells spiking frequency is reduced compared to in wild-type mice
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• mice exhibit increased spontaneous GABAergic inhibitory postsynaptic currents compared with wild-type mice
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• mice exhibit larger miniature inhibitory postsynaptic currents compared with wild-type mice
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growth/size/body
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• beginning at P7 in some mice
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• beginning at P7 in some mice
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