hearing/vestibular/ear
• elevated threshold and reduced amplitudes
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behavior/neurological
• mice fail to improve performance in rotating rod trials in contrast to wild-type animals
(J:137190)
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• in a non-rotating rod test, equilibrium times of mutants are lower than controls in the first trial, but not the 2nd or 3rd trial
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• mutants exhibit poor performance in a motor training test with a rotarod, and are unable to improve their performance
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• tapping of the hindpaws; phenotype is much less severe than in Grid2ho-Nancy mice
• mutants exhibit increased stumbling frequency in a motor coordination test
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cellular
• mitochondria lengths in Purkinje cells are 1.3-1.6 um, compared to lengths of 0.8-1.0 um in wild-type animals
• often, points where mitochondria become constricted are observed in mutants but not in wild-type mitochondria, suggestive of impaired fission
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nervous system
• numerous clusters of naked spines are located in the molecular layer around secondary branches and spiny branchlets of Purkinje cell dendrites
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• mitochondria lengths in Purkinje cells are 1.3-1.6 um, compared to lengths of 0.8-1.0 um in wild-type animals
• often, points where mitochondria become constricted are observed in mutants but not in wild-type mitochondria, suggestive of impaired fission
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• Purkinje cell dendrites have ectopic spines, some of which are naked and bore very long postsynaptic differentiations directly apposed to astrocytic processes
• occasional appositions between a Purkinje cell dendritic spine and two parallel fiber varicosities
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• some granule cell pyknotic nuclei in the granule cell layer
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• although most Purkinje cells are innervated by a single climbing fiber, about 19-20% of cells show innervation by more than one climbing fiber
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• mismatching between the length of the presynaptic active zones, in parallel fiber varicosities, and the postsynaptic differentiations in Purkinje cell dendritic spines
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