Analysis Tools
behavior/neurological
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• mice show a decreased latency to fall off the accelerating rod
• in the walking beam test, mice exhibit an increase in the number of slips of a hind leg per crossing of the beam
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nervous system
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• at P12, in cerebellum lobule 7, the number of Purkinje cells is reduced and cells are not properly arranged in the Purkinje cell layer but are dispersed in the molecular layer
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• at 3 months of age, gaps are seen in the Purkinje cell layer of cerebellum lobule 7, suggesting Purkinje cell loss in this area
• however, mice do not show overt Purkinje cell loss in other areas of the cerebellum
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• Purkinje cells exhibit a highly abnormal dendritic tree in organotypic slice cultures; the dendritic tree is reduced in size and branch development and the primary dendritic branches appear thickened and have only short secondary side branches
• juvenile mutants exhibit Purkinje cells with dendritic abnormalities in the area of lobule 7 of the cerebellum, most pronounced at P12 and P18, with mild changes in the area in adults
• at P12, stem dendrites appear thickened and are shortened and the distal dendrites are missing or reduced in size
• at P18, size of dendrites increases, however dendrites are still thickened
• at P28, there is a further increase in dendritic size, but dendrites are still thicker and devoid of small side branches
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• at P12, in cerebellum lobule 7, the number of Purkinje cells is reduced
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| spinocerebellar ataxia type 14 | DOID:0050964 |
OMIM:605361 |
J:218354 | |
