behavior/neurological
• mice exhibit reduced motor learning on a rotarod compared with mice containing only one of the transgenes
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• mice spend more time in the inner regions of an open field compared with wild-type mice
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• some mice exhibit spastic head movements unlike wild-type mice
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• beginning at 2 to 3 months of age
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• when lowered onto a horizontal pen, mice fail to walk along it and fall with severely affected mice unable to sit on the pen unlike wild-type mice
• as early as 9 weeks of age, mice exhibit impaired performance on a rotarod compared with mice containing only one of the transgenes
• however, mice treated with doxycycline for 5 months exhibit improved rotarod performance
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• in an open field test
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nervous system
• at 21 months, Purkinje cells are smaller than in wild-type mice
• however, mice treated with doxycycline treatment from 2 months of age exhibit normal Purkinje cell size
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• at 21 months, the cerebellar molecular layer is thinner than in wild-type mice
• however, mice treated with doxycycline treatment from 2 months of age exhibit normal cerebellar molecular layer thickness
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growth/size/body
• mice exhibit reduced body weight compared with Tg(tetO-ATXN3)2904Olri mice
• however, treatment with doxycycline at 9 weeks of age restores weight
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
Machado-Joseph disease | DOID:1440 |
OMIM:109150 |
J:154079 |