Mouse Genome Informatics
tg
    Tg(Prnp-HTT*82Q)52Caro/0
involves: C3H * C57BL/6
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
mortality/aging
• mutants die around 1 year of age

growth/size
• signs of deterioration become apparent in males at 6 months of age
• weight loss begins at around 4 months of age and continues progressively with disease

behavior/neurological
• at 8 months of age, mutants exhibit signs of cognitive impairment in the fear conditioning paradigm
• on the second day of a fear conditioning paradigm, mutants exhibit a reduction in the amount of time spent freezing compared to controls, indicating impaired contextual memory
• on the third day of a fear conditioning paradigm, mutants show an even larger deficit in cue-dependent memory
• mutants exhibit frequent clasping when suspended by tail
• mutants exhibit impaired rotarod performance beginning at 4 months of age
• toward end stage of disease, mutants have a hunched posture
• mutants become hyperactive after 5 months of age, which is even more pronounced at 7 months of age
• toward end stage of disease, mutants become hypoactive and are immobile

nervous system
• at 8 months of age, mutants exhibit a 15.35% decrease in whole brain size indicating brain atrophy

Mouse Models of Human Disease
OMIM IDRef(s)
Huntington Disease; HD 143100 J:179364