Glial mismanagement of neuromuscular junction structure and function in ALS

old_uid14721
titleGlial mismanagement of neuromuscular junction structure and function in ALS
start_date2017/11/21
schedule15h30-16h30
onlineno
location_infosalle 04
detailsHosted by Christian Lobsiger
summaryAmyotrophic 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. Interestingly, NMJ vulnerability to denervation is highly correlated to motor unit subtypes where those with fast- fatigable properties are the most vulnerable. Despite the importance of NMJ denervation, NMJ vulnerability and resistance remain under-characterized and the mechanisms involved ill defined. NMJ synaptic transmission, morphological stability and repair are actively regulated by Perisynaptic Schwann Cells (PSCs), glial cells at this synapse. 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. Overall, these results suggest that PSC alterations may contribute to NMJ vulnerability in ALS.
responsiblesOliviero