General Information

Mouse: CB57BL/6

Expression of the human wild type alpha-synuclein protein under the control of the Thy1 promoter

Endogenous mouse alpha synuclein: Yes

Corresponding human genotype: Autosomal dominant mutation in PD patients (PARK1)

Transgene insertion:  not reported

References: Chesselet 2012 ; Martin 2014

Transgene expression

  • 6-12 months : strong expression of the mutated hasynA53T throughout the brain (cortex, olfactory bulb, striatum, cerebellum, diencephalon as well as a strong expression) as well as in spinal cord and motor neuron.

Neurodegeneration

  • Age-related loss of TH-positive neurons in the SN and the striatum reaching 60% at endstage (12 months)
  • Age-related degeneration of striatal and cerebellum interneurons
  • 8-12 months: age-related degeneration of cerebral cortex (strong), hippocampus (moderate), striatal and cerebellum interneurons, and motor thalamus.

Dopamine Homeostasis

  • Not reported

Inclusions

  • 6 months: aggregates observed mainly in the spinal cord brainstem, hippocampus and cerebellum.
  • 8-12 months: inclusions detected in all brain regions

Motor Behaviours

  • 2-4 months: normal spontaneous motor activity compared to control littermates (PMID 24325796)
  • 6 months : motor deficits (spasticity, tremors)
  • 8 months: rigidity, bradykinesia and gait abnormalities
  • 12 months : complete immobility and death (usually between 10-12 months).

Response to L-DOPA treatment

  • Not reported

Non motor Behaviours

  • Not reported

Electrophysiology

  • Not reported

Neuroinflammation

  • Not reported

 

 

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