Phenotypes Associated with This Genotype

Genotype
MGI:6814676

• Allelic Composition: Tg(Thy1-SNCA*)#Ztzh/0
• Genetic Background: involves: C57BL/6J

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

behavior/neurological

abnormal behavior ( J:313970 )

• in the pole test, young mice spend less time hesitating and take action earlier to descend compared to controls, however these differences reverse at 16 months of age and mice have a longer latency to climb down

increased anxiety-related response ( J:313970 )

• 3-month-old mice exhibit a shorter latency to fall in the grid performance test, but this is not seen in older mice and thus may be due to anxiety

increased thigmotaxis ( J:313970 )

• in the open field test, 16-month-old or older mice spend more time in the peripheral area near the wall

• the residence time and mobile time in the center of the open field test start to decline at 9 months of age

limb grasping ( J:313970 )

• mice show clasping in the tail suspension test , with mean distances of hind paws declining by 9 and 16 months of age

impaired coordination ( J:313970 )

• aged mice show worse performance on the rotarod

abnormal vertical activity ( J:313970 )

• mice exhibit increased rearing at 3 months of age but less rearing time at 16 months of age

decreased locomotor activity ( J:313970 )

• in the open field test, 16-month-old or older mice move less

increased locomotor activity ( J:313970 )

• young mice cross more lines and have more active time in the open field test which decreases at 16 months

digestive/alimentary system

decreased feces water content ( J:313970 )

• mice excrete drier stool from a very early age

constipation ( J:313970 )

• mice excrete drier stool from a very early age indicating constipation

growth/size/body

decreased body weight ( J:313970 )

• mice weigh less than wild-type mice after 9 months of age

homeostasis/metabolism

decreased nervous system dopamine level ( J:313970 )

• concentration of dopamine and its metabolites DOPAC and HVA is decreased in the striatum at 9 months of age

muscle

muscle weakness ( J:313970 )

• worse rotarod performance and myodynamia suggest declining muscle strength after 9 months of age

nervous system

loss of dopaminergic neurons ( J:313970 )

• mice exhibit loss of TH+ cells (dopaminergic neurons) after 9 months of age but not at 3 months indicating age-dependent progressive nigrostriatal dopaminergic degeneration

abnormal synapse morphology ( J:313970 )

• mice show a reduction in the number of synaptic density areas in the striatum indicating age-dependent striatal synaptic degeneration

abnormal synaptic vesicle number ( J:313970 )

• only sparse vesicles are seen in the striatum of 16-month-old mice

neuron degeneration ( J:313970 )

• mice exhibit loss of TH+ cells (dopaminergic neurons) after 9 months of age but not at 3 months indicating age-dependent progressive nigrostriatal dopaminergic degeneration

alpha-synuclein inclusion body ( J:313970 )

• mice show early alpha-synucleinopathy, with Ser129 phosphorylated alpha-synuclein seen at 3 months, aggregations seen in the cortex at 9 months, accumulation of granular Ser129 phosphorylated alpha-synuclein in the spinal cord, and age-dependent aggregation of the insoluble alpha-synuclein 1-130 fragment in the substantia nigra and striatum

• however, typical Lewy bodies are missing from the cytoplasm of the motor neurons in the anterior horn

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Parkinson's disease 1		DOID:0060367	OMIM:168601	J:313970