Analysis Tools
behavior/neurological
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• by 12 months of age, almost all mice show abnormal clasping response
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• mice fail the ledge test by 12 months of age
• mice exhibit progressive incoordination and ultimately an abnormal gait at 16-20 months of age
• mice show a shorter latency to fall times on the rotarod by 12 months of age, with most of these mice having difficulty moving their limbs rapidly and have lowered and flattened hips
• however, mice do not develop tremors, seizures, rigidity, or kyphosis
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• mice exhibit an abnormal gait at 16-20 months of age
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nervous system
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• Bergmann glia shows swelling of radial processes
• Bergmann glia radial processes show more GFAP immunoreactivity
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• shrinkage of Purkinje cell bodies
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• degeneration and dropout of Purkinje cells becomes apparent with disease progression
• mice exhibit vacuole formation in Purkinje cell dendrites in the molecular layer of the cerebellum, dendritic spine thinning, and Bergmann glia processes ensheathing Purkinje cells that lack subcellular organelles, indicating dark cell degeneration of Purkinje cells
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• mice show reductions in Purkinje cell dendritic arborization
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• molecular layer is atrophied
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• uptake of glutamate by cultured Bergmann glia is reduced
• uptake of glutamate in cerebellar slices form 9 month old mice is reduced by 20-25%
• glutamate uptake capacity is reduced by approximately 40% in cerebellar synaptosomes
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| spinocerebellar ataxia type 7 | DOID:0050958 |
OMIM:164500 |
J:113150 | |
