Phenotypes Associated with This Genotype

Genotype
MGI:5008740

• Allelic Composition: Tg(PDGFB-UCHL1*I93M)HWada/?
• Genetic Background: involves: C57BL/6J

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

nervous system

abnormal nervous system morphology ( J:128045 )

• 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

abnormal striatum morphology ( J:128045 )

• nigro-striatal fibers show reduced TH-immunoreactivity

decreased dopaminergic neuron number ( J:128045 )

• 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

decreased locomotor activity ( J:128045 )

• modest but significant decrease in activity during the dark phase at 19-21 weeks of age

increased locomotor activity ( J:128045 )

• increase in locomotion during the dark phase at 11-13 weeks of age in the home cage

homeostasis/metabolism

decreased nervous system dopamine level ( J:128045 )

• decrease in striatal dopamine concentration