What is the primary mechanism of neurotoxicity in Prion diseases?
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Correct answer: Conformational change of PrPC to the protease-resistant PrPSc
Prion diseases occur when the normal cellular prion protein (PrPC) undergoes a post-translational change into an abnormal beta-pleated sheet conformation (PrPSc). This abnormal protein is resistant to proteolysis and induces further misfolding of PrPC.
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