Mouse Genome Informatics
tg
    Tg(Prnp-HTT*82Q,KRT14-EGFP)C63Dbo/0
Not Specified
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
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
• by one third at end-stage

homeostasis/metabolism
• at end-stage

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