Figure 1. Role of posttranslational processing of PrP^C in the formation of prion aggregates.

Fibrillar prion strains such as mCWD preferentially incorporate underglycosylated PrP^C (blue) that is released from the cell surface by ADAM-10 into plaques that form in association with HS in the parenchyma. Subfibrillar prion stains, such as RML, preferentially incorporate glycosylated PrP^C into diffuse synaptic deposits of PrP^Sc (red) without a HS scaffold. Genetic ablation of N-linked glycosylation sites (N) can switch in nonfibrillar strain pathology from synaptic diffuse deposition to plaque-like deposition of PrP^Sc in the parenchyma.