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Phenotypes Associated with This Genotype
involves: C3H * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Prnp-SNCA*A53T)83Vle mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
• 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
DO ID OMIM ID(s) Ref(s)
autosomal dominant Parkinson disease 1 DOID:0060367 OMIM:168601

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
MGI 6.08
The Jackson Laboratory