General Information
Mouse: C57BL6/J
Mice in which exon 7 of the DJ-1 gene were deleted leading to the absence of DJ-1 protein in homozygous DJ-1-/- mice. Male and female mice are fertile, show no changes in weight gain compared to wt mice, and comparable life span.
Endogenous DJ-1: No
Corresponding human genotype: Autosomal recessive genetic deletion in the DJ-1 gene causing a loss-of-function of the protein and leading to early-onset Parkinson’s disease (PARK7).
Mutated gene: DJ-1
References: Manning-Bog 2007
Neurodegeneration
- 3-4 months: No apparent changes in the number TH-positive neurons. MPTP treatment induces a more severe loss of TH-positive terminals in the striatum but not of TH-positive neurons in the SN.
Increased DAT function is observed (causing higher MPTP uptake and toxicity)
Dopamine Homeostasis
- 3-4 months: No apparent changes DA and DA metabolites levels are observed. Increased DA reuptake is observed. MPTP treatment induces a more severe depletion of DA and DA metabolites in KO mice compared to wt mice (likely linked to increased DAT function in KO mice).
Inclusions
- Not reported
Motor Behaviours
- 3-4 and 12 months: No motor deficits are observed (inverted grid, stride length).
- 12 months: slight increase in forepaw faults is observed.
Response to dopaminergic treatment
- Not reported
Non motor Behaviours
- Not reported
Electrophysiology
- Not reported
Neuroinflammation
- 3-4 months: No apparent astroglial activation is obsevred in the SN following MPTP treatment
Updated 25/04/2018