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  • Richard Robitaille,  Professor , Neuroscience Department ,Montreal University, Canada

    Title: Glial mismanagement of neuromuscular structure and function in ALS

     Neuroscience Seminar Series:
    Friday, June 8th. 2018, 11:30 am, Salle des conférences (3rd Floor), Centre Universitaire des Saints-Pères, 45 rue des Saints-Pères, 75006 Paris


    Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disease with a complex etiology. The destruction of neuromuscular junctions (NMJs) is an early event that leads to muscle weakness and paralysis due to the denervation and retraction of nerve terminals from striated muscles. Despite the importance of NMJ denervation, NMJ vulnerability and resistance remain under-characterized and the mechanisms involved ill defined. We focus on the role of Perisynaptic Schwann Cells (PSCs), glial cells at this synapse, since they regulate NMJ synaptic transmission, morphological stability and repair. I will discuss ex vivo and in vivo experiments to illustrate that neuromuscular changes are precocious, with a strong influence of the local environment in the outcome of NMJ denervation in ALS. Data will be presented to suggest that targeting PSCs help restore NMJ innervation and muscle functions.



    Host : Claire Legay