Analysis Tools
mortality/aging
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• recovered at a frequency (20.5%) slightly less than the expected Mendelian ratio at two weeks of age
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nervous system
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• reduced number of proliferating granule cell precursors (GCPs) in the secondary fissure in the posterior cerebellum at P8
• loss of proliferating GCPs is concentrated in areas where the basement membrane (BM) is disrupted
• normal proliferation of GCPs in the cerebella-derived culture
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• scores of granule cell precursors (GCPs) abnormally protrude into the subarachnoid space between the midbrain and cerebellum at E17
• laminin-labeled basement membrane (BM) is fragmented at E15 and E17
• loss of the pial BM in the cerebellum starting from around P8
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• reduced anterior-posterior extent of the mutant adult cerebellum
• basic laminar structure of the anterior cerebellum, encompassing lobules I-V, is completely lost
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• obvious defects in the organization of anterior lobules I-V in P7 and P1 cerebella
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• adjacent lobules, such as lobules VIII and IX, are fused
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• patches of granule cells are randomly scattered throughout the white matter
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• abnormal Bergmann glial network correlates with breaches of the BM in the cerebellum at P8
• highly disorganized radial glial processes, with some of their endfeet protruding into the meninges in the mutant cerebellum at E15
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• loosely aligned Purkinje cells, with some abnormally distributing at the cerebellar surface at P1
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• GCPs ectopically embed within the cortex at P1
• ectopic granule cell differentiation near the broken BM at P8
• many granule cells ectopias at the cerebellar surface in the posterior regions of adult cerebella and along the fusion lines of adjacent lobules
• normal extent of migration of granule cells and granule cell-glia interactions at P10 before the loss of the BM
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• decreased depth of the fissures
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• both the cerebellar vermis and the two lateral hemispheres lack clear fissures on the surface
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• disappearance of lobules IV and V in the anterior vermis
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• smaller cerebellum in adult mice
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behavior/neurological
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• take approximately twice as long to cross the elevated balance beam
• dramatic increase in the number of foot slips
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cellular
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• reduced number of proliferating granule cell precursors (GCPs) in the secondary fissure in the posterior cerebellum at P8
• loss of proliferating GCPs is concentrated in areas where the basement membrane (BM) is disrupted
• normal proliferation of GCPs in the cerebella-derived culture
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