Analysis Tools
nervous system
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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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astrocytosis
(
J:120070
)
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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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behavior/neurological
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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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trunk curl
(
J:120070
)
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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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muscle
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• at 12 weeks, frequent paroxysmal bursts of dystonic hindlimb retraction are observed
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growth/size/body
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• males and females weigh 17% and 25%, respectively, less than wild-type
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immune system
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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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hematopoietic system
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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 | |
