nervous system
N |
• Purkinje cell exhibit normal passive electrical properties
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• fewer spike discharges following injection of depolarizing currents 800 pA without a change in interspike interval
• spontaneous spike rate is smaller than in wild-type mice
• however, response to depolarizing currents 400 pA is normal
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• Purkinje cells exhibit reduced sodium current amplitude compared with wild-type cells
• however, Purkinje cell activation and inactivation properties
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• following injection of depolarizing currents 800 pA with strong adaptation during action potential (AP) firing and a large depolarization plateau after AP burst and shorter AP train duration
• reduced total number of evoked AP without any changes in AP firing frequency above 800 pA current injection
• smaller AP amplitude at 800 pA current injection
• smaller percentage of spontaneous AP firing Purkinje cells
• however, response to 400 pA current injection is normal and mean interspike interval is normal at 400 and 800 pA current injection
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• altered cortical electroencephalogram when moving with increased alpha-, beta- and delta-wave power with reduced theta-wave power
• altered electromyogram median power
• however, brain wave patter when not moving is normal
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• increased
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• increased
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• increased
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• reduced
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behavior/neurological
• severe head tremor during movement after 3 to 4 weeks of age
• body vibration
• however, treatment with gabapentin or propranolol improves head tremor frequency as does Purkinje cell-specific Arf5 expression
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• impaired performance on a rotarod
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• fewer spike discharges following injection of depolarizing currents 800 pA without a change in interspike interval
• spontaneous spike rate is smaller than in wild-type mice
• however, response to depolarizing currents 400 pA is normal
|