behavior/neurological
• mutants show impaired performance on the rotarod at 11 months of age, but not earlier, and are unable to improve their performance during successive days
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nervous system
• at 16 months of age, but not at 11 months, mutants exhibit a reduction in the density of Purkinje cells, indicating degeneration
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• at 16 months of age, but not at 11 months, mutants exhibit a reduction in the dendritic arbor of Purkinje cells
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• progressive formation of ubiquitin and proteasome-immunoreactive nuclear inclusions composed of the N-terminal fragment of the mutant protein
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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:65411 |