Mouse Genome Informatics
tg
    Tg(PDGFB-UCHL1*I93M)HWada/?
involves: C57BL/6J
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
nervous system
• decrease in striatal dopamine concentration
• cytoplasmic inclusions in the form of large aggregates are seen in the remain portion of the substantia nigra at 20 weeks of age
• these inclusions are ubiqutin and UCHL1 positive but SNCA negative
• electron dense-core vesicles are seen in the cytoplasm of about 30% of nigral neurons
• nigro-striatal fibers show reduced TH-immunoreactivity
• 30% reduction in TH-positive neurons at 20 weeks of age but not at 12 weeks of age, compared to non-transgenic controls

behavior/neurological
• increase in locomotion during the dark phase at 11-13 weeks of age in the home cage
• modest but significant decrease in activity during the dark phase at 19-21 weeks of age

homeostasis/metabolism
• decrease in striatal dopamine concentration

Mouse Models of Human Disease
OMIM IDRef(s)
Parkinson Disease 5, Autosomal Dominant; PARK5 613643 J:128045