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Phenotypes Associated with This Genotype
Genotype
MGI:5286128
Allelic
Composition
Tg(Prnp-HTT*82Q,KRT14-EGFP)C63Dbo/0
Genetic
Background
Not Specified
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
mortality/aging
• mice die by 8 months of age when they are unable to acquire food or water
• average endpoint is 247 days

nervous system
• by half at end-stage
• contains large inclusions
• profound hippocampal degeneration in the CA1 and CA2 regions with partial degeneration in the CA3 region
• contain Htt+ cytoplasmic inclusions

behavior/neurological
• mice exhibit dyskinesia with ataxia-like movement compared with control mice
• beginning at 4 months and worsening to end-stage by 8 months of age
• at 4 months
• in mice displaying dyskinesia on a rotarod
• at 3 months, mice exhibit a moderate rotarod deficit that progresses to severe by 6 months of age compared with control mice
• aged mice exhibit a 10- to 50-fold deficit in latency to fall on a rotarod compared with control mice

growth/size/body
• by one third at end-stage

homeostasis/metabolism
• at end-stage

Mouse Models of Human Disease
OMIM ID Ref(s)
Huntington Disease; HD 143100 J:173756


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
04/26/2016
MGI 6.03
The Jackson Laboratory