Mouse Genome Informatics
tg
    Tg(Prnp-ATN1)150Dbo/?
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
• mice begin dying as early as 10 weeks, many are dead by 50 weeks

behavior/neurological
• beginning at 1-2 months
• involuntary tremors beginning at 1-2 months
• progressive ataxia beginning at 1-2 months
• loss of limb coordination beginning at 1-2 months
• beginning at 1-2 months
• decreased breeding efficiency
• seizures are observed in some animals

nervous system
• seizures are observed in some animals
• inclusion bodies observed in the dentate nucleus of the cerebellum of 12 month old mice
• a few inclusions are observed in the heart

Mouse Models of Human Disease
OMIM IDRef(s)
Dentatorubral-Pallidoluysian Atrophy; DRPLA 125370 J:70362