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Phenotypes Associated with This Genotype
Genotype
MGI:5635158
Allelic
Composition
Tg(HTT*)NXwy/0
Genetic
Background
involves: FVB/NJ
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(HTT*)NXwy mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• mice show increased immobility in the forced swim test at 5 months of age, suggesting presence of depression-like behavior
• mice at 10-11 months of age are frequently found lying upside down in their home cage, poorly groomed
• in the accelerating rotarod test, mice exhibit progressive impairment at 2 and 6 months of age and by 8 months of age, mice can no longer run on it
• in the open field test, mice show impairment in vertical plane entry at 8, but not 2, months of age
• mice show sustained retroflex of head and neck indicating cervical dystonia at 11 months of age
• in the open field test, mice show impairment in horizontal distance traveled, and horizontal speed at 8, but not 2, months of age
• gait abnormalities are seen at 8 months of age
• starting around 10 months of age, mutants show frequent spontaneous falls
• mice at 10-11 months of age are frequently found lying upside down in their home cage
• mice show frequent abnormal head movements at 11 months of age, consisting of smooth but non-rhythmic swinging of the head and upper body indicating choreiform movement, and sustained retroflex of head and neck indicating cervical dystonia
• onset of abnormal head movements is around 7 months of age and increases progressively

endocrine/exocrine glands
• in 10 month old mice

growth/size/body
• mice exhibit significant weight loss at 10 and 11 months of age
• both males and females are heavier than wild-type mice at 2 and 6 months of age

immune system
• gene expression profiling indicates progressive neuroinflammation

muscle
• mice show sustained retroflex of head and neck indicating cervical dystonia at 11 months of age
• atrophy, reduction of muscle fiber caliber and aberrant central localization of nuclei within the fiber of quadriceps muscle

nervous system
• gene expression profiling indicates progressive neuroinflammation
• progressive forebrain weight loss at 6-10 months of age, with a 34% forebrain weight loss at 10 months of age
• about 40% loss of striatal medium spiny neurons at 10 months of age
• striatal volume loss in 10 month old mice
• cortical volume loss in 10 month old mice
• reactive astrocytes with hypertrophic processes are seen in the striatum and cortex at 10 months of age
• nuclear inclusions containing aggregated mutant HTT are seen in both the cortex and striatum by 6 months of age and in other brain regions such as the hippocampus, thalamus, cerebellum, and brain stem at 6-10 months of age
• however, cytoplasmic aggregates are not seen
• local field potential recordings in the cortex and dorsal striatum show synchronous, short duration, large-amplitude events in the low gamma range in 7-9 month old mice
• differences in gamma frequency events are already seen in 16-18 week old mice and progressively worsen with age
• however, epileptic activity is not seen is cortical local field potential recording

reproductive system
• in 10 month old mice
• age-dependent infertility starting around 5 months of age

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Huntington's disease DOID:12858 OMIM:143100
J:219890


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
10/08/2019
MGI 6.14
The Jackson Laboratory