Mouse Genome Informatics
tg
    Tg(PDGFB-SNCA)4Ema/0
involves: C57BL/6 * DBA/2
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
behavior/neurological
• during the spatial learning period of the water maze with the platform submerged, mutants exhibit show performance deficits compared with controls
• treatment of mice with the mGluR5 antagonist MPEP show improved performance in the water maze
• in the water maze probe test in which the platform was removed on the last day, mutants show differences in the entrances and passes into the target zone compared to controls; again, treatment with MPEP resulted in similar behavior to controls
• mutants are impaired in ability to negotiate a pole apparatus as evidenced by a significantly longer T-turn time
• treatment of mice with a mGluR5 antagonist MPEP results in an improvement in T-turn time
• mice show deficits in motor performance in the rotorod test compared to wild-type mice

nervous system
• prominent cytoplasmic and nuclear inclusions immunoreactive to human alpha-synuclein are observed by 2 months of age; these are most frequent in neurons of the deep neocortex, CA3 region of the hippocampus, olfactory bulb and occasionally the substantia nigra
• in 9-11 month old mice, larger inclusions composed of fine granular material are seen, and contain clear vacuoles
• alpha-synuclein protein accumulates in synapses and neurons throughout the brain, including the neuropil of presynaptic terminals, neocortex, hippocampus, cerebellum, substantia nigra, limbic system, and olfactory regions, and inclusion bodies form in neurons in the deeper layers of the neocortex and in axons
• alpha-synuclein protein accumulation is also seen in the glial cells along the white matter tracts in the colliculus, thalamus, brainstem, hippocampus, and cerebellum

Mouse Models of Human Disease
OMIM IDRef(s)
Dementia, Lewy Body; DLB 127750 J:60638 , J:137490 , J:166985
Parkinson Disease 1, Autosomal Dominant; PARK1 168601 J:60638 , J:166985