Analysis Tools
behavior/neurological
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• impaired performance on an accelerating rotarod is first seen at 18 days of age
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• waddling gait
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nervous system
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• sporadic pyknotic nuclei are seen after the onset of locomotor abnormalities; however, cerebellar foliation and laminated cortical structure are normal
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• the number of parallel fiber-Purkinje cell synapses is reduced with about 50% of proximal dendrite Purkinje cell spines and 80% of distal dendrite Purkinje cell spines lacking synaptic contacts
• many of those parallel fiber-Purkinje cell synapses that do form have postsynaptic densities that are longer than the active zone
• at P60 to P68 only 53% of homozygotes had Purkinje cells with a 1 to 1 relationship with innervating climbing fibers unlike in wild-type mice where all Purkinje cells have a 1 to 1 relationship by P21 to P30
• the density of climbing fiber innervation is increased compared to wild-type and penetrated further into the molecular layer (87 +/- 1% of the thickness compared to 79 +/- 3% in wild-type mice)
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• the amplitudes of Purkinje cell parallel fiber excitatory postsynaptic currents are reduced
• some Purkinje cell climbing fiber excitatory postsynaptic current amplitudes are smaller and these are often associated with slower rise times
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• at P21- to P30, direct depolarization of Purkinje cells induces long term depression in wild-type mice but not in homozygotes
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