Analysis Tools
mortality/aging
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• die by approximately 4 weeks
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• born at expected Mendelian ratio
• more than half (16% of total progeny) survive to 3 weeks
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growth/size/body
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• reduced body weight at postnatal day 20 (P20)
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behavior/neurological
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• severe ataxia at P20-P25
• unable to maintain their balance and show poorly coordinated movement
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nervous system
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• delayed granule cell proliferation peak
• reduced BrdU-positive cells at P3 and P7, and relatively increase at P10
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• smaller cerebellar primordia at E13.5
• two primordia are barely attached at the medial region, which fail to further expand at E15.5
• the medulla oblongata and rostral spinal cord remain open at E15.5
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• smaller lateral cerebellar hemispheres with fewer folia starting at P3
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• defects in the formation of hippocampal commissure in P0 and adult mice
• callosal axons fail to cross the midline
• axons swirl into longitudinal neuromas called Probst bundles (callosal agenesis)
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• reduced septum in the forebrain at E13.5 and E15.5
• midline glia is preserved at the cortical edge, but not in the medial region at E15.5
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• enlarged cerebral ventricles accompanied with shrinkage of the thalamus
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• defects in the formation of corpus callosum in P0 and adult mice
• callosal axons fail to cross the midline
• axons swirl into longitudinal neuromas called Probst bundles (callosal agenesis)
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• enlarged cerebral ventricles accompanied with shrinkage of the thalamus
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• smaller lateral cerebellar hemispheres with fewer folia starting at P3
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• greatly reduced dendritic arborization of Purkinje cells at P10 and adulthood
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• greatly reduced number of Purkinje cells at P10 and adulthood
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• severely reduced or missing central vermis region
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• absent cerebellum in the most severely affected mice
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cellular
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• delayed granule cell proliferation peak
• reduced BrdU-positive cells at P3 and P7, and relatively increase at P10
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