[SEMINAIRE] Delphine Bohl, "Modeling Amyotrophic Lateral Sclerosis with iPSC: motor neuron intrinsic defects and rôles of inflammatory cells towards their degeneration"

Summary : Amyotrophic Lateral Sclerosis (ALS) is a rapidly fatal motor neuron degenerative disorder with no curative treatment. To find new therapeutic targets, we focused our interests on comparing mechanisms involved in motor neuron degeneration and the role of inflammatory cells towards their degeneration. For that, we differentiate induced pluripotent cell (iPSC) derived from patients with different ALS forms (genetic and sporadic) as well as control subjects and isogenic controls, into motor neurons, microglial cells and macrophages. During my presentation, I will present common and divergent intrinsic phenotypes we observed in ALS MNs, and the interest to distinguish microglial cells reacting to degeneration in the spinal cord from macrophages reacting at the periphery to axonal degeneration.