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Allele Symbol Allele Name Allele ID |
Tg(tetO-ATXN3)2904Olri transgene insertion 2904, Olaf Riess MGI:4410556 |
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| Summary |
4 genotypes
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• in some neuronal cells of the cerebral cortex by 20 month old mice
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| N |
• mice exhibit a normal gait and performance on a rotarod
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
|
• 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
• mice exhibit impaired performance on a rotarod compared with mice containing only one of the transgenes
• however, mice treated with doxycycline exhibit improved rotarod performance
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• in an open field test, especially in inner areas
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• at 20 months
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• in some neuronal cells of the cerebral cortex by 1 month old mice
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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 | |
|
|
| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
|
• mice spend more time in the inner regions of an open field than wild-type mice
|
|
• some mice exhibit spastic head movements unlike wild-type mice
|
|
• beginning at 2 to 3 months of age
|
|
• 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
• mice exhibit impaired performance on a rotarod compared with mice containing only one of the transgenes
• however, mice treated with doxycycline exhibit improved rotarod performance
|
|
• at 20 months, mice exhibit an altered gait with smaller distance between steps compared with wild-type mice
|
|
• in an open field test
|
|
• at 20 months
|
|
• in some neuronal cells of the cerebral cortex by 1 month old mice
|
|
• mice exhibit reduced body weight compared with Tg(tetO-ATXN3)2904Olri mice
• however, treatment with doxycycline at 9 weeks of age restores weight
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Machado-Joseph disease | DOID:1440 |
OMIM:109150 |
J:154079 | |
|
|
| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
|
• mice exhibit reduced motor learning on a rotarod compared with mice containing only one of the transgenes
|
|
• mice spend more time in the inner regions of an open field compared with wild-type mice
|
|
• some mice exhibit spastic head movements unlike wild-type mice
|
|
• beginning at 2 to 3 months of age
|
|
• 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
|
|
• in an open field test
|
|
• 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
|
|
• 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
|
|
• mice exhibit reduced body weight compared with Tg(tetO-ATXN3)2904Olri mice
• however, treatment with doxycycline at 9 weeks of age restores weight
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Machado-Joseph disease | DOID:1440 |
OMIM:109150 |
J:154079 | |
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO) |
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last database update 05/14/2024 MGI 6.23 |
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