behavior/neurological
• begins at 24 weeks of age
|
• mice become less alert and less exploratory at about 24 weeks of age, regardless of whether they previously showed hyperactivity
• decrease in activity lasts 4-6 weeks and progresses to locomotor deterioration and akinesia
|
hyperactivity
(
J:50173
)
• begins at 24 weeks of age
|
• begins at 24 weeks of age
|
• 10 of 24 mice show stereotypy beginning at 24 weeks of age, consisting of generalized hyperactive behavior, including unidirectional rotations, backflips, and excessive grooming
|
• lack of response to sensory stimuli precedes death
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limb grasping
(
J:50173
)
• mice exhibit feet clasping following suspension by their tails at 8 weeks of age and from then on throughout life
|
growth/size/body
• mice do not gain weight when they become hyperactive
|
nervous system
astrocytosis
(
J:50173
)
• fibrillary astrocytosis, which is most evident in hypokinetic and akinetic mice
• astocytosis is most prominent in the striata
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• neuronal loss, which is most evident in hypokinetic and akinetic mice
• neuronal loss is most prominent in the striata, but is also seen in the hippocampus, thalamus, and cerebral cortex
• however, no evidence of degeneration of Purkinje or granule cells
|
• neuronal intranuclear inclusions are seen in neurons of the striatum, cerebral cortex, hippocampus, thalamus, and cerebellum, and some Purkinje cells; less than 1% of neurons from the striatum show intranuclear inclusions
|
renal/urinary system
• urinary incontinence is frequently seen at 24 weeks of age
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
Huntington's disease | DOID:12858 |
OMIM:143100 |
J:50173 |