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Mécanismes de transmission synaptique in vivo de la SOD1 humaine mal repliée

Chef d'équipe 
  • Neil Cashman, University of British Columbia
  • ALS Society of Canada

Aperçu du projet

SOD1 is a protein that is known to misfold and cause ALS. Recent evidence suggests that this misfolded protein might spread between brain cells (neurons) at their communication points (synapses). This might explain how pathology spreads around the central nervous system in disease. There is evidence that this could happen in cells in a dish, but it has never been demonstrated in a whole living animal. Furthermore, we do not know how it happens. Dr. Cashman and his team have developed a novel model in which we show that this does in fact occur in a living animal when we put human SOD1 into fruit fly neurons. The beauty of the fruit fly (Drosophila) model is that we can look at just one particular synapse and show unequivocally that the protein has moved. Moreover, genetic tools exist in Drosophila which allow the team to manipulate any part of the cell machinery to test which parts are required for this protein movement to occur. This will allow us to understand how the protein moves, which provides an avenue for identifying new therapeutic targets to stop the spread of pathology