Mouse Genome Informatics
tg
    Tg(Prnp-SNCA*A53T)83Vle/0
involves: C3H * C57BL/6
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
behavior/neurological
• develop the severe and complex motor impairment leading to paralysis and death that is seen in homozygous transgenics, however onset is delayed from 16 months of age to 22-28 months of age (J:76657)

nervous system
• develop a similar neurodegenerative disease that is observed in homozygous transgenic mice, however onset is delayed from 16 months of age to 22-28 months of age (J:76657)
• mice develop some inclusions that are composed of both tau and alpha-synuclein (J:127923)
• about 25% of diseased mutants accumulate abundant tau-positive threads, grains and spheroids in regions of the pons, midbrain, and spinal core
• rare perikaryal tau inclusions with morphology of a pre-tangle are seen
• less frequent tau inclusions are present in another 25% of symptomatic mutants but none are seen in the rest 50% of diseased mutants
• mice exhibit formation of abundant alpha-synuclein inclusions

Mouse Models of Human Disease
OMIM IDRef(s)
Parkinson Disease 1, Autosomal Dominant; PARK1 168601 J:76657