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Phenotypes Associated with This Genotype
Genotype
MGI:5569536
Allelic
Composition
Tg(CMV-HTT*89Q)ATag/0
Genetic
Background
involves: FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• begins at 24 weeks of age
• decrease in activity progresses to akinesia
• 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
• 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
• 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
• focal gliosis in the hippocampus, thalamus, and cerebral cortex
• fibrillary astrocytosis, which is most evident in hypokinetic and akinetic mice
• astocytosis is most prominent in the striata
• 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


Contributing Projects:
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
10/15/2019
MGI 6.14
The Jackson Laboratory