behavior/neurological
• mutants do not display ataxia in standard environments; when challenged (with a runway test or rotarod test) coordination is severely disturbed compared to controls
• mice display a shaky tremor which usually worsens during movement; controls do not show any tremors
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nervous system
• dendritic calcium transients associated with complex spikes (synaptic response) upon climbing fiber stimulation is altered in mutants compared to controls; peak amplitudes are enhanced by ~80% over wild-type
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hearing/vestibular/ear
N |
• at 4-8 months, homozygotes exhibit normal cochlear morphology, as well as normal hearing and cochlear OHC function, as assessed by baseline auditory brainstem evoked responses and DPOAEs, respectively
• at 3 weeks after exposure to a moderate noise trauma, homozygotes exhibit a similar loss of cochlear hair cells to wild-type mice, with no significant differences in permanent threshold shifts of ABR or DPOAE amplitudes
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