Analysis Tools
normal phenotype
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• mice do not exhibit myoclonic jerks, dystonia or locomoter retardation, survive to a normal age, and are fertile
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Allele Symbol Allele Name Allele ID |
Drd1tm2Jcd targeted mutation 2, John Drago MGI:3707260 |
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| Summary |
3 genotypes
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• mice do not exhibit myoclonic jerks, dystonia or locomoter retardation, survive to a normal age, and are fertile
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• at P2 mice have a small or absent milk spot and most die during the first postnatal week with some surviving to P19
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• at P4
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• at P4
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• some distortion in the normal relationship of the forebrain and hippocampus
• islands of Calleja are absent
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• forebrain is smaller and volumetrically reduced by about 40% in the striatum with no change in cortical thickness
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• hypercellular stratum with frequent condensed nuclear bodies
• mice have an increase apoptotic striatum cells
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• some distortion in the normal relationship of the forebrain and hippocampus
• hippocampus is displayed anteriorly
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• increase in reactive gliosis in the lateral stratum and along the corpus callosum
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• neuropeptides substance P and dynorphin are absent
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• falls are more myoclonic jerks than ataxia and are sometimes locomotor-activated
• at less than P6, all mice exhibit myoclonic jerks compared to 2 of 11 normal mice
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• mice fail to right after spontaneous falls
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• frequent falls at P2 some due to dystonia while other falls are erratic, violent, intrusive and associated with attempted movement or tactile stimulation
• falls are more myoclonic jerks than ataxia
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• mice have an abnormal posture when at rest
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• falls are more myoclonic jerks than ataxia and are sometimes locomotor-activated
• at less than P6, all mice exhibit myoclonic jerks compared to 2 of 11 normal mice
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• falls are more myoclonic jerks than ataxia and are sometimes locomotor-activated
• at less than P6, all mice exhibit myoclonic jerks compared to 2 of 11 normal mice
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• at P2 mice exhibit periodic breathing that is resolved in older pups
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• after 4 weeks, approximately 12% smaller width of the entire brain compared to controls
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• at 34 weeks brains weigh 15% less than wild-type
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• striatal atrophy and secondary enlargement of the lateral ventricles are observed at 4 weeks in 1 mouse and in another 5 at 6 weeks
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• dramatic reduction in striatal size in the second month
• striatal volume is decreased by 60% with a 65% loss in cell numbers and 17% reduction in cell density
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• striatal atrophy and secondary enlargement of the lateral ventricles are observed at 4 weeks in 1 mouse and in another 5 at 6 weeks
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• decrease in dentate gyrus cell number
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• hippocampus volume is decreased
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• cortex volume is significantly decreased without a change in cell density or total cell number
• cortical thickness is decreased at 4,6, 8 and 30 weeks
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• astrocytes had a reactive astrocyte morphology with large cell bodies and complex ramified cellular processes
• the number of reactive astrocytes (GFAP+) increases over time with a 20-fold higher number of GFAP+ cells at 9 weeks, then reduces at later time points but always remains high than in controls
• reactive astrocytes are seen in the hippocampus and cortex
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• at 5 weeks, handling-induced seizures occur
• spontaneous seizures were recorded in 2 of 5 mice
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• activated microglia (CD11b+) are present in the cortex, hippocampus, thalamus and caudate putamen at 3 weeks but numbers decrease over time
• at 5 weeks less microglial reactivity is seen in the thalamus and no activated microglia are seen in the cortex or hippocampus
• at 21 weeks mice are free of microglia
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• interictal electroencephalogram (EEG) is abnormal
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• at 4 weeks, hindlimb clasping is subtle
• at 12 weeks, frequent paroxysmal bursts of dystonic hindlimb retraction are observed
• at 14 weeks, mice sustain hindlimb clasping with trunk flexion
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• at 12 weeks, frequent paroxysmal bursts of dystonic hindlimb retraction are observed
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• at 14 weeks trunk flexion associated with hindlimb clasping
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• at 6 to 9 weeks and 16 to 21 weeks, there is a decrease in chewing and shifting while total rearing is increased
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• at 6 to 9 weeks but not at 16 to 21 weeks, mice show increases in distance covered, time spent moving and speed of movement
• increase in sniffing
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• at 5 weeks, handling-induced seizures occur
• spontaneous seizures were recorded in 2 of 5 mice
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• at 12 weeks, frequent paroxysmal bursts of dystonic hindlimb retraction are observed
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• males and females weigh 17% and 25%, respectively, less than wild-type
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• activated microglia (CD11b+) are present in the cortex, hippocampus, thalamus and caudate putamen at 3 weeks but numbers decrease over time
• at 5 weeks less microglial reactivity is seen in the thalamus and no activated microglia are seen in the cortex or hippocampus
• at 21 weeks mice are free of microglia
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• activated microglia (CD11b+) are present in the cortex, hippocampus, thalamus and caudate putamen at 3 weeks but numbers decrease over time
• at 5 weeks less microglial reactivity is seen in the thalamus and no activated microglia are seen in the cortex or hippocampus
• at 21 weeks mice are free of microglia
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Huntington's disease | DOID:12858 |
OMIM:143100 |
J:120070 | |
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 04/16/2024 MGI 6.23 |
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