nervous system
N |
• normal cerebellar foliation and lamination of cortical structures
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• reduced number of vGluT2+ puncta in the cerebella
• climbing fiber terminals penetrate to a shallower depth than in wild-type mice
• however, transforming Purkinje cells with a wild-type form of the gene re-establishes climbing fiber synapse elimination
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• reduced complex spikes from Purkinje cells
• reduced climbing fiber-evoked afterhyperpolarization and suppression of spontaneous spikes
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• whole-cell patch-clamp recordings show reduced one-to-one relationship of Purkinje cells with climbing fibers and smaller amplitude of strongest climbing fiber excitatory postsynaptic currents
• however, transforming Purkinje cells with a wild-type form (but not one lacking a domain required for interaction with C1ql1) of the gene re-establishes increases amplitude of strongest climbing fiber excitatory postsynaptic currents
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• simultaneously activating parallel fibers and climbing fibers for 2 min at 1 Hz in the current-clamp mode fails to develop long term depression
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behavior/neurological
N |
• mice exhibit no ataxia and normal motor coordination on the rotor-rod test
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• severely impaired adaptation of the horizontal optokinetic response
• however, horizontal optokinetic response prior to training is normal
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