• hES from control and affected patients with Huntingtin mutations (different lengths of CAG triplet): differentiation into cortical / striatal neurons
  • iPS cells from control and affected patients with Huntingtin mutations (different lengths of CAG triplet): cell lines from fibroblasts could be differentiated into cortical / striatal neurons or glial cells.
  • Primary cortical or striatal neuron cultures with or without astrocytes
    Treatment with quinolinate, glutamate, 3-nitropropionate
  • Primary cortical or striatal neuron cultures of neurons infected with AAV or LV vectors coding huntingtin gene (different lengths of CAG triplet)
READ-OUT

Cell survival, morphological changes, protein measurement, etc.

 

  • Striatal cell degeneration: injections of quinolinate (or other glutamate agonists) or chronic systemic 3NP
  • Transgenic models of progressive degeneration (KI140CAG mouse, BACHD rat)
  • Progressive striatal degeneration: injection of lentiviral /AAV vectors encoding fragments of mutated huntingtin gene
READ-OUT

Neuroinflammation and glucose metabolism PET imaging, MRI, MR spectroscopy, 3D -histology, motor and cognitive behavior, etc.

 

  • Excitotoxic striatal lesions (Macaque): injections of quinolinate (or other glutamate agonists) or chronic systemic 3NP
  • Progressive striatal degeneration: injection of lentiviral /AAV vectors encoding fragments of mutated huntingtin gene
READ-OUT Neuroinflammation and glucose metabolism PET imaging, MRI, MR spectroscopy, histology, motor and cognitive behavior, etc.

 

  • Clinical investigation Center, Biological resource center, neurology units, stratified cohorts of patients, coordination of the French National Reference Centre for Huntington's disease and the Huntington's disease group of the European Network for Rare Diseases
READ-OUT PET and MRI imaging, Markers of dopaminergic system and neuroinflammation, platforms of movement analysis, specific cognitive tools

 

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