behavior/neurological
N |
• mutants exhibit normal exploratory activity, pain sensitivity, motor coordination on a rotarod, and spatial memory despite impaired LTP
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• in a wheel running test, 4-6 week old mutants show fewer number of steps per minute and reduced total horizontal displacement of the hindlimb foot
• in the presence of a sensory perturbation, locomotion remains poor and elevations of the hindfoot are induced
• the feet of aged (18-24 months) mutants slip more often that those of wild-type in the runway test
• aged mutants spend less time on a rotarod than wild-type
• in a wheel running test, aged mutants frequently fall after hindfoot elevations in the presence of a sensory perturbation
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nervous system
• severe impairment of Purkinje cell firing properties, with a doubling of the spontaneous simple spike firing rate, a halving of the duration of complex spike, and a 70-80% reduction in the duration of the simple spike pause after the complex spike
• however, excitatory and synaptic transmission and short-term plasticity are normal in cerebellar slices
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• induction of long-term potentiation (LTP) is impaired in the dentate gyrus; mutants show a reduced posttetanic potentiation that decays progressively over time
• however, LTP is induced normally in CA1 region
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