General Information
Mouse: CB57BL/CH3
Expression of the mutant (E46K) human alpha-synuclein protein under the control of the mouse Prion Protein (PrP) gene promoter
Endogenous mouse alpha synuclein: Yes
Corresponding human genotype: Autosomal dominant mutation in PD patients (PARK1/PARK4)
Transgene insertion: not specified
References: Emmer 2011
Transgene expression
- 3 months: expression of transgene is observed in the brain and the spinal cord.
Neurodegeneration
- No loss of TH-positive neurons observed
Dopamine Homeostasis
- Not reported
Inclusions
- 12 months: accumulation of abnormal alpha-synuclein is observed in the cortex and the spinal cord with high molecular weight aggregates containing alpha-synuclein phosphorylated at serine 129.
- No inclusions are observed in the SN at any time.
Motor Behaviours
- Starting at 16 and within 29 months: E46K mice develop asymmetric and slowly progressing hind leg paralysis. Changes also include neglect of grooming, weight loss and reduced ambulation. No retraction of hind limb is observed when mice are held by the tail.
- From 2-19 months: no changes in gait and wire hang testing detected in pre-symptomatic mice.
Response to L-DOPA treatment
- Not reported
Non motor Behaviours
- Not reported
Electrophysiology
- Not reported
Neuroinflammation
- Not reported