Prion Protein Misfolding, Strains, and Neurotoxicity: An Update from Studies on Mammalian Prions
Table 1
Cofactors enhancing PrPC conversion in vitro.
Cofactor
Experimental setting
Results
Refs.
Pentosan polysulfate (PPS)
Cell-free conversion assay
Hamster and mouse [35S] GPI(-) PrPC seeded with brain derived PrPres from infected hamsters (263 K) and mice (87 V)
(i) PPS increases the rate of formation and the yield of [35S] PrPres (ii) PPS facilitates conversion of both Mo and SHa [35S] GPI(-) PrPC at different temperatures
(i) Stochastic de novo formation of PrPSc molecules from unseeded purified substrates (ii) Both amplified Sc237 or 139H PrPSc and de novo PrPSc molecules cause scrapie in inoculated Syrian hamsters
(i) In vitro generated recPrPres (ii) recPrPres propagates its PK-resistant conformation to endogenous PrPC (iii) recPrPres causes bona fide prion disease in wild-type mice