Analysis Tools|
Allele Symbol Allele Name Allele ID |
Tg(Prnp-tTA)F959Sbp transgene insertion F959, Stanley B Prusiner MGI:2445786 |
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| Summary |
7 genotypes
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• in mice administered doxycycline for 2 weeks, restoration of wild-type drinking behavior
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• increased duration of regaining ethanol-induced loss or righting response, increase in duration of pentobarbital-induced loss or righting response
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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 |
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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
• 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 | |
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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 than 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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• at 20 months, mice exhibit an altered gait with smaller distance between steps compared with wild-type mice
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• in an open field test
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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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• 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 | |
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| ♀ | 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
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• in an open field test
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• 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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• 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 | |
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|
| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• increased exploratory behavior observed in open and closed arms of plus maze and open field test
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• mice exhibit an enhanced spatial learning by locating the visible and hidden platforms of the Morris water maze in less time than controls
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• mice exhibit an accelerated retrieval of spatial memory by remaining in target quadrant of probe trial (platform removed from water maze)
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• mice are more active than controls
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• increased number of rearing events in the open field test
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• mice exhibit increased sensitivity to handling-induced seizures
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• mice exhibit increased sensitivity to handling-induced seizures
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• I/O function, a measure of synaptic strength, in untrained mice is significantly impaired relative to controls
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• LTP induced in hippocampal slices is higher than controls, however synapse numbers are not increased
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• mice exhibit seizures of 3 to 21 episodes during 24 hours each lasting 15 to 40 seconds
• seizures occur mainly during periods of increased delta activity regardless of doxycycline treatment as adults to silence the transgene
• most seizures occur during the light period (resting-sleeping)
• however, doxycycline treatment between E1 and P15 prevents seizures
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• mice display abnormal electroencephalogram with frequent spikes of high amplitudes unlike wild-type mice
• delta waves predominant unlike in wild-type mice where theta waves are more representatives
• seizures occur mainly during periods of increased delta activity regardless of doxycycline treatment as adults to silence the transgene
• however, doxycycline treatment between E1 and P15 prevents high amplitude spikes and restores prevalence of theta waves
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• mice exhibit seizures of 3 to 21 episodes during 24 hours each lasting 15 to 40 seconds
• seizures occur mainly during periods of increased delta activity regardless of doxycycline treatment as adults to silence the transgene
• most seizures occur during the light period (resting-sleeping)
• however, doxycycline treatment between E1 and P15 prevents seizures
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| autosomal dominant nocturnal frontal lobe epilepsy 3 | DOID:0060684 |
OMIM:605375 |
J:145855 | |
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 12/10/2024 MGI 6.24 |
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