mDJ-1 KO/3 Mouse

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

 

 

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