Prion propagation

Prion propagation involves conversion of PrPc to PrPSc 795... 

All three of these predictions from this minimal model are manifest in the etiology of prion disease an inversely proportional relationship between PrPc expression and prion incubation period in transgenic mice predisposition by relatively subtle mutations in the protein sequence and a requirement for molecular homogeneity between PrPSc and PrPc for efficient prion propagation [4, 5, 20]. It is clear that a full understanding of prion propagation will require knowledge both of the structure of PrPc and PrPSc and of the mechanism of conversion between them. 

Weissmann, C. A unified theory of prion propagation. Nature 352 679-683,1991. 

Asante, E. A. et al. BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein. EMBO J. 21 6358-6366,2002. 

Norstrom, E. M., and Mastrianni, J. A. (2005). The AGAAAAGA palindrome in PrP is required to generate a productive PrPSc-PrPC complex that leads to prion propagation./. Biol. Chem. 280, 27236-27243. 

Several NMR studies were carried out to gain insight into the molecular basis for the conversion of the PrP " into the pathogenic conformer, PrP . They mainly involve the study of prion-derived peptides believed to be essential for prion propagation, although other studies also include the investigation of the whole prion protein ° and prion mutants. 

Example Conformational Autocatalysis (A Model of Prion Propagation)... 

E. Malolepsza et al., Theoretical model of prion propagation A misfolded protein induces misfolding. Proc. Natl. Acad. Sci. USA 102, 7835-7840 (2005)... 

Peretz D, Williamson RA, Kaneko K, Vergara J, Leclerc E, Schmitt-Ulms G, Mehlhorn IR, Legname G, Wormald MR, Rudd PM, Dwek RA, Burton DR, Prusiner SB (2001) Antibodies inhibit prion propagation and clear cell cultures of prion infectivity. Nature 412 739-743. 

M. F. Tuite Sowing the protein seeds of prion propagation. Science 289,556 (2(XX)). 

Green KM, Browning SR, Seward TS et al (2008) The elk PRNP codon 132 polymorphism controls cervid and scrapie prion propagation. J Gen Virol 89 598-608... 

Abstract Here we review the known strain profiles of various prion diseases of animals and humans, and how transgenic mouse models are being used to elucidate basic molecular mechanisms of prion propagation and strain variation and for... 

Telling GC, Scott M, Mastrianni J, Gabizon R, Torchia M, Cohen FE, DeArmond SJ, Prusiner SB (1995) Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another protein. Cell 83 79-90... 

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