In vivo models of Parkinson’s disease – Mammalian models

Genetic models

MitoPark mouse

Evidence indicates an important role of mitochondrial dysfunction in sporadic Parkinson’s disease (PD) and familial forms of PD are often linked to genes involved in the mitochondrial function. In addition, a large number of neurotoxins that affect the mitochondrial respiration chain induce the death of dopaminergic cells.

It has also been shown that dopaminergic neurons of PD patients contain an elevated number of somatic mutations in mitochondrial DNA (mtDNA), which affect the mitochondrial respiration chain.

To investigate whether mitochondrial dysfunction plays a role in the etiology of PD causing neuronal cell death (primary role) or if it is a consequence of degenerating neurons (secondary role), a conditional knock-out mouse with a disruption in a gene fundamental for mtDNA maintenance, the mitochondrial transcription factor A (Tfam), specifically in midbrain dopaminergic neurons, has been developed: the MitoPark mouse.

Tfam is involved in mitochondrial biogenesis and mtDNA maintenance; it stabilizes mtDNA and regulates copy number. In the absence of Tfam a progressive deficiency in mitochondrial respiratory chain is observed followed by cell death.

To generate the MitoPark mouse model, knock-in mice expressing Cre recombinase under the control of the dopaminergic transporter (DAT) were crossed with mice bearing  loxP recombination sites flanking the Tfam gene (TfamloxP). In this way Tfam deletion is directed only in cells expressing DAT.


SpeciesGene Knock outMouse genotypeNeurodegeneration (Y/N)Link

Updates 2018-11-27

2 thoughts on “MitoPark mouse

  1. Kamal Maher

    My name is Kamal Maher, and I am a researcher in the Jesse Goldberg lab at Cornell University. We are considering purchasing mouse models of Parkinson’s for our studies concerning motor deficits in PD.

    1) Is it possible to purchase a genetically HEMI-Parkinsonian mouse?
    2) In any case, how soon could we acquire 10 MitoPark mice?

    1. parkinsonuser Post author

      Hemi-parkinson rats can be obtained from Charles River. I am no certain 6-ohda mice are commercially available.
      Mitopark mice have been developed at the Max Plank Institute. You should contact them to have more information on the model availability. Contat names can be found on the reference link present in the Mitopark model page.


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