mortality/aging
• mice survive up to approximately 14 weeks
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behavior/neurological
• motor defects
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nervous system
• mice show progressive degeneration of granule cell neurons such that the inner granule layer is largely devoid of granule cell neurons at P48
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• P95 mice have severely atrophic cerebellum with the inner granule layer largely devoid of granule cell neurons at P48
• however, cerebellar phenotypes are less severe than in homozygous Tubb4aJit mice
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• reduction in white matter tracts such as corpus callosum, anterior commissures, and striatal fascicles at P95
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• mice show progressive degeneration of myelin with reduced myelin at P48 and P95, however reduction in myelin is not as severe as in in homozygous Tubb4aJit mice
• cerebellum is severely demyelinated at P95
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