The Elusive Role of the Prion Protein and the Mechanism of Toxicity in Prion Disease
Fig 2
A role for intracellular PrPC retention in neuronal dysfunction.
(A) PrPC on the plasma membrane (PM) influences the activity of neurotransmitter receptors, ion channels, and signaling complexes with which it interacts. (B) Owing to retention in transport organelles (ER/Golgi), misfolded/aggregated PrPC sequesters the interacting protein in intracellular compartments, leading to loss of normal function on the cell membrane [6]. Intracellular retention might also cause the complex to function abnormally and generate a toxic signal.