Prion protein fragments

Other phenomena. Prion proteins, the mutated forms of which cause Creutzfeldt-Jacob disease, also appear to interact with metal ions. Spectra of Cu(H) bound to various mutant proteins have indicated anomalous binding behaviour.331 Hydroxyl radical generation during the interaction of mutant forms of a prion protein fragment with Fe(II) has also been reported.332... 

Forloni G, Angeretti N, Chiesa R, Monzani E, Salmona M, Bugiani O, Tagliavini F (1993) Neurotoxicity of a prion protein fragment. Nature 362 543... 

Perovic, S., Pergande, G., Ushijima, H., Kelve, M., Forrest, J., and Muller, W.E. (1995). Flupirtine partially prevents neuronal injury induced by prion protein fragment and lead acetate. Neurodegenemtion 4, 369-374. 

Florio T, Paludi D, Villa V, Principe DR, Corsaro A, Millo E, Damonte G, D Arrigo C, Russo C, Schettini G, Aceto A (2003) Contribution of two conserved glydne residues to fibrillogenesis of the 106-126 prion protein fragment. Evidence that a soluble variant of the 106-126 peptide is neurotoxic. J Neurochem 85 62-72... 

Villa V, Tonelli M, Thellung S, Corsaro A, Tasso B, Novell F, et al. Efficacy of novel acridine derivatives in the inhibition of hPrP90-231 prion protein fragment toxicity. Neurotox Res 2011 19(4) 556-74. 

D.D. Laws, H-M.L. Bitter, K, Liu, H.L. Ball, K. Kaneko, H. Wille, F.E, Cohen, S.B. Prusiner, A. Pines, and D.E. Wemmer, Solid-state NMR Studies of the Secondary Structure of a Mutant Prion Protein Fragment of 55 Residues that Induces Neurodegeneration. Proc. Natl. Acad. Sci. U.S.A., 98,11686-11690,2001. 

Umland, T. C., Taylor, K. L., Rhee, S., Wickner, R. B., and Davies, D. R. (2001). The crystal structure of the nitrogen regulation fragment of the yeast prion protein Ure2p. Proc. Natl. Acad. Sci. USA 98, 1459-1464. 

Horiuchi, M., Baron, G. S., Xiong, L. W., and Caughey, B. (2001). Inhibition of interactions and interconversions of prion protein isoforms by peptide fragments from the C-terminal folded domain./. Biol. Chem. 276, 15489-15497. 

Ragg, E., Tagliavini, F., Malesani, P., Monticelli, L., Bugiani, O., Forloni, G., and Salmona, M. (1999). Determination of solution conformations of PrP106-126, a neurotoxic fragment of prion protein, by 1H NMR and restrained molecular dynamics. Eur.J. Biochem. 266, 1192-1201. 

J. El Khoury, S.E. Hickman, C.A. Thomas, H. Suzuki, T. Kodama and S.C. Silverstein, Class A scavenger receptors mediate adhesion of microglia to a neurotoxic fragment of prion protein, Conference Proceedings of Biomedicine 98, Washington DC, 1998. 

Leclerc E, Liemann S, Wildegger G, Vetter SW, Nilsson F (2000) Selection and characterization of single chain Fv fragments against murine recombinant prion protein from a synthetic human antibody phage display library. Hum Antibodies 9 207-214. 

Fig. 5 Models of prion replication, (a) The template assistance model predicts that a PrPSo monomer is more stable than PrPc, but is kinetically inaccessible. In the rare event that a PrPSo monomer is created spontaneously (or provided exogenously), it can template the misfolding of another PrPc molecule by direct interaction. The dashed line shows that the newly created PrPSc monomer can act as another seed to formation of PrPSc. (b) The nucleation polymerization model predicts that barrier to prion protein conversion is the formation of a nucleus in which the protein adopts a PrPSo-like structure. The formation of such a low order aggregate is not favored however, once it has formed, polymerization from a pool of PrPc molecules can take place efficiently. Fragmentation of the polymer increases the number of ends for the recruitment of PrPc monomers...

Kazmirski SL, Alonso DOV, Cohen FE, Prusiner SB, Daggett V (1995) Theoretical-studies of sequence effects on the conformational properties of a fragment of the prion protein implication for scrapie formation. Chem Biol 2 305... 

James TL, Liu H, Ulyanov NB, FarrJones S, Zhang H, Donne DG, Kaneko K, Groth D, Mehlhom I, Prusiner SB, Cohen FE (1997) Solution stmcture of a 142-residue recombinant prion protein corresponding to the infectious fragment of the scrapie isoform. Proc Natl Acad Sci USA 94 10086... 

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