A closer look at prion strains

References

• DeArmond SJ. Alzheimer’s disease and Creutzfeldt-Jakob disease: overlap of pathogenic mechanisms. Curr Opin Neurol 1993; 6:872 - 81; http://dx.doi.org/10.1097/00019052-199312000-00008; PMID: 7904883
   PubMed Web of Science ®Google Scholar
• DeArmond SJ, McKinley MP, Barry RA, Braunfeld MB, McColloch JR, Prusiner SB. Identification of prion amyloid filaments in scrapie-infected brain. Cell 1985; 41:221 - 35; http://dx.doi.org/10.1016/0092-8674(85)90076-5; PMID: 3922627
   PubMed Web of Science ®Google Scholar
• Prusiner SB. Novel proteinaceous infectious particles cause scrapie. Science 1982; 216:136 - 44; http://dx.doi.org/10.1126/science.6801762; PMID: 6801762
   PubMed Web of Science ®Google Scholar
• Prusiner SB. Prions. Proc Natl Acad Sci U S A 1998; 95:13363 - 83; http://dx.doi.org/10.1073/pnas.95.23.13363; PMID: 9811807
   PubMed Web of Science ®Google Scholar
• Prusiner SB. Molecular biology of prion diseases. Science 1991; 252:1515 - 22; http://dx.doi.org/10.1126/science.1675487; PMID: 1675487
   PubMed Web of Science ®Google Scholar
• Collinge J. Prion diseases of humans and animals: their causes and molecular basis. Annu Rev Neurosci 2001; 24:519 - 50; http://dx.doi.org/10.1146/annurev.neuro.24.1.519; PMID: 11283320
   PubMed Web of Science ®Google Scholar
• Weissmann C. The state of the prion. Nat Rev Microbiol 2004; 2:861 - 71; http://dx.doi.org/10.1038/nrmicro1025; PMID: 15494743
   PubMed Web of Science ®Google Scholar
• Aguzzi A, Polymenidou M. Mammalian prion biology: one century of evolving concepts. Cell 2004; 116:313 - 27; http://dx.doi.org/10.1016/S0092-8674(03)01031-6; PMID: 14744440
   PubMed Web of Science ®Google Scholar
• Caughey B. Interactions between prion protein isoforms: the kiss of death?. Trends Biochem Sci 2001; 26:235 - 42; http://dx.doi.org/10.1016/S0968-0004(01)01792-3; PMID: 11295556
   PubMed Web of Science ®Google Scholar
• Perrier V, Kaneko K, Safar J, Vergara J, Tremblay P, DeArmond SJ, et al. Dominant-negative inhibition of prion replication in transgenic mice. Proc Natl Acad Sci U S A 2002; 99:13079 - 84; http://dx.doi.org/10.1073/pnas.182425299; PMID: 12271119
   PubMed Web of Science ®Google Scholar
• Deleault NR, Lucassen RW, Supattapone S. RNA molecules stimulate prion protein conversion. Nature 2003; 425:717 - 20; http://dx.doi.org/10.1038/nature01979; PMID: 14562104
   PubMed Web of Science ®Google Scholar
• Deleault NR, Harris BT, Rees JR, Supattapone S. Formation of native prions from minimal components in vitro. Proc Natl Acad Sci U S A 2007; 104:9741 - 6; http://dx.doi.org/10.1073/pnas.0702662104; PMID: 17535913
   PubMed Web of Science ®Google Scholar
• Deleault NR, Walsh DJ, Piro JR, Wang F, Wang X, Ma J, et al. Cofactor molecules maintain infectious conformation and restrict strain properties in purified prions. Proc Natl Acad Sci U S A 2012; 109:E1938 - 46; http://dx.doi.org/10.1073/pnas.1206999109; PMID: 22711839
   PubMed Web of Science ®Google Scholar
• Cohen FE, Pan KM, Huang Z, Baldwin M, Fletterick RJ, Prusiner SB. Structural clues to prion replication. Science 1994; 264:530 - 1; http://dx.doi.org/10.1126/science.7909169; PMID: 7909169
   PubMed Web of Science ®Google Scholar
• Pan KM, Baldwin M, Nguyen J, Gasset M, Serban A, Groth D, et al. Conversion of alpha-helices into beta-sheets features in the formation of the scrapie prion proteins. Proc Natl Acad Sci U S A 1993; 90:10962 - 6; http://dx.doi.org/10.1073/pnas.90.23.10962; PMID: 7902575
   PubMed Web of Science ®Google Scholar
• Peretz D, Williamson RA, Matsunaga Y, Serban H, Pinilla C, Bastidas RB, et al. A conformational transition at the N terminus of the prion protein features in formation of the scrapie isoform. J Mol Biol 1997; 273:614 - 22; http://dx.doi.org/10.1006/jmbi.1997.1328; PMID: 9356250
   PubMed Web of Science ®Google Scholar
• Zhang H, Stockel J, Mehlhorn I, Groth D, Baldwin MA, Prusiner SB, et al. Physical studies of conformational plasticity in a recombinant prion protein. Biochemistry 1997; 36:3543 - 53; http://dx.doi.org/10.1021/bi961965r; PMID: 9132005
   PubMed Web of Science ®Google Scholar
• Soto C, Castilla J. The controversial protein-only hypothesis of prion propagation. Nat Med 2004; 10:Suppl S63 - 7; http://dx.doi.org/10.1038/nm1069; PMID: 15272271
   PubMed Web of Science ®Google Scholar
• Laurent M. Prion diseases and the ‘protein only’ hypothesis: a theoretical dynamic study. Biochem J 1996; 318:35 - 9; PMID: 8761449
   PubMed Web of Science ®Google Scholar
• Tanaka M, Chien P, Naber N, Cooke R, Weissman JS. Conformational variations in an infectious protein determine prion strain differences. Nature 2004; 428:323 - 8; http://dx.doi.org/10.1038/nature02392; PMID: 15029196
   PubMed Web of Science ®Google Scholar
• Morales R, Abid K, Soto C. The prion strain phenomenon: molecular basis and unprecedented features. Biochim Biophys Acta 2007; 1772:681 - 91; http://dx.doi.org/10.1016/j.bbadis.2006.12.006; PMID: 17254754
   PubMed Web of Science ®Google Scholar
• Telling GC, Parchi P, DeArmond SJ, Cortelli P, Montagna P, Gabizon R, et al. Evidence for the conformation of the pathologic isoform of the prion protein enciphering and propagating prion diversity. Science 1996; 274:2079 - 82; http://dx.doi.org/10.1126/science.274.5295.2079; PMID: 8953038
   PubMed Web of Science ®Google Scholar
• Bessen RA, Kocisko DA, Raymond GJ, Nandan S, Lansbury PT, Caughey B. Non-genetic propagation of strain-specific properties of scrapie prion protein. Nature 1995; 375:698 - 700; http://dx.doi.org/10.1038/375698a0; PMID: 7791905
   PubMed Web of Science ®Google Scholar
• DebBurman SK, Raymond GJ, Caughey B, Lindquist S. Chaperone-supervised conversion of prion protein to its protease-resistant form. Proc Natl Acad Sci U S A 1997; 94:13938 - 43; http://dx.doi.org/10.1073/pnas.94.25.13938; PMID: 9391131
   PubMed Web of Science ®Google Scholar
• Collinge J. Medicine. Prion strain mutation and selection. Science 2010; 328:1111 - 2; http://dx.doi.org/10.1126/science.1190815; PMID: 20508117
   PubMed Web of Science ®Google Scholar
• Giles K, Glidden DV, Patel S, Korth C, Groth D, Lemus A, et al. Human prion strain selection in transgenic mice. Ann Neurol 2010; 68:151 - 61; http://dx.doi.org/10.1002/ana.22104; PMID: 20695008
   PubMed Web of Science ®Google Scholar
• Pattison IH, Millson GC. Scrapie produced experimentally in goats with special reference to the clinical syndrome. J Comp Pathol 1961; 71:101 - 9; PMID: 13733383
   PubMed Web of Science ®Google Scholar
• Chesebro B. BSE and prions: uncertainties about the agent. Science 1998; 279:42 - 3; http://dx.doi.org/10.1126/science.279.5347.42; PMID: 9441410
   PubMed Web of Science ®Google Scholar
• Fraser H, Dickinson AG. Scrapie in mice. Agent-strain differences in the distribution and intensity of grey matter vacuolation. J Comp Pathol 1973; 83:29 - 40; http://dx.doi.org/10.1016/0021-9975(73)90024-8; PMID: 4199908
   PubMed Web of Science ®Google Scholar
• Kascsak RJ, Rubenstein R, Merz PA, Carp RI, Wisniewski HM, Diringer H. Biochemical differences among scrapie-associated fibrils support the biological diversity of scrapie agents. J Gen Virol 1985; 66:1715 - 22; http://dx.doi.org/10.1099/0022-1317-66-8-1715; PMID: 3926951
   PubMed Web of Science ®Google Scholar
• Bessen RA, Marsh RF. Biochemical and physical properties of the prion protein from two strains of the transmissible mink encephalopathy agent. J Virol 1992; 66:2096 - 101; PMID: 1347795
   PubMed Web of Science ®Google Scholar
• Medori R, Tritschler HJ, LeBlanc A, Villare F, Manetto V, Chen HY, et al. Fatal familial insomnia, a prion disease with a mutation at codon 178 of the prion protein gene. N Engl J Med 1992; 326:444 - 9; http://dx.doi.org/10.1056/NEJM199202133260704; PMID: 1346338
   PubMed Web of Science ®Google Scholar
• Lugaresi E, Montagna P, Baruzzi A, Cortelli P, Tinuper P, Zucconi M, et al. [Familial insomnia with a malignant course: a new thalamic disease]. Rev Neurol (Paris) 1986; 142:791 - 2; PMID: 3823713
   PubMed Web of Science ®Google Scholar
• Goldfarb LG, Petersen RB, Tabaton M, Brown P, LeBlanc AC, Montagna P, et al. Fatal familial insomnia and familial Creutzfeldt-Jakob disease: disease phenotype determined by a DNA polymorphism. Science 1992; 258:806 - 8; http://dx.doi.org/10.1126/science.1439789; PMID: 1439789
   PubMed Web of Science ®Google Scholar
• Monari L, Chen SG, Brown P, Parchi P, Petersen RB, Mikol J, et al. Fatal familial insomnia and familial Creutzfeldt-Jakob disease: different prion proteins determined by a DNA polymorphism. Proc Natl Acad Sci U S A 1994; 91:2839 - 42; http://dx.doi.org/10.1073/pnas.91.7.2839; PMID: 7908444
   PubMed Web of Science ®Google Scholar
• Bessen RA, Marsh RF. Identification of two biologically distinct strains of transmissible mink encephalopathy in hamsters. J Gen Virol 1992; 73:329 - 34; http://dx.doi.org/10.1099/0022-1317-73-2-329; PMID: 1531675
   PubMed Web of Science ®Google Scholar
• Bruce ME. Scrapie strain variation and mutation. Br Med Bull 1993; 49:822 - 38; PMID: 8137131
   PubMed Web of Science ®Google Scholar
• Fraser H. Diversity in the neuropathology of scrapie-like diseases in animals. Br Med Bull 1993; 49:792 - 809; PMID: 8137129
   PubMed Web of Science ®Google Scholar
• Collinge J, Sidle KC, Meads J, Ironside J, Hill AF. Molecular analysis of prion strain variation and the aetiology of ‘new variant’ CJD. Nature 1996; 383:685 - 90; http://dx.doi.org/10.1038/383685a0; PMID: 8878476
   PubMed Web of Science ®Google Scholar
• Parchi P, Castellani R, Capellari S, Ghetti B, Young K, Chen SG, et al. Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease. Ann Neurol 1996; 39:767 - 78; http://dx.doi.org/10.1002/ana.410390613; PMID: 8651649
   PubMed Web of Science ®Google Scholar
• Khalili-Shirazi A, Summers L, Linehan J, Mallinson G, Anstee D, Hawke S, et al. PrP glycoforms are associated in a strain-specific ratio in native PrPSc. J Gen Virol 2005; 86:2635 - 44; http://dx.doi.org/10.1099/vir.0.80375-0; PMID: 16099923
   PubMed Web of Science ®Google Scholar
• Safar J, Wille H, Itri V, Groth D, Serban H, Torchia M, et al. Eight prion strains have PrP(Sc) molecules with different conformations. Nat Med 1998; 4:1157 - 65; http://dx.doi.org/10.1038/2654; PMID: 9771749
   PubMed Web of Science ®Google Scholar
• Wadsworth JD, Hill AF, Joiner S, Jackson GS, Clarke AR, Collinge J. Strain-specific prion-protein conformation determined by metal ions. Nat Cell Biol 1999; 1:55 - 9; http://dx.doi.org/10.1038/9030; PMID: 10559865
   PubMed Web of Science ®Google Scholar
• Zanusso G, Farinazzo A, Prelli F, Fiorini M, Gelati M, Ferrari S, et al. Identification of distinct N-terminal truncated forms of prion protein in different Creutzfeldt-Jakob disease subtypes. J Biol Chem 2004; 279:38936 - 42; http://dx.doi.org/10.1074/jbc.M405468200; PMID: 15247220
   PubMed Web of Science ®Google Scholar
• Zou WQ, Capellari S, Parchi P, Sy MS, Gambetti P, Chen SG. Identification of novel proteinase K-resistant C-terminal fragments of PrP in Creutzfeldt-Jakob disease. J Biol Chem 2003; 278:40429 - 36; http://dx.doi.org/10.1074/jbc.M308550200; PMID: 12917418
   PubMed Web of Science ®Google Scholar
• Caughey B, Raymond GJ, Bessen RA. Strain-dependent differences in beta-sheet conformations of abnormal prion protein. J Biol Chem 1998; 273:32230 - 5; http://dx.doi.org/10.1074/jbc.273.48.32230; PMID: 9822701
   PubMed Web of Science ®Google Scholar
• Tanaka M, Collins SR, Toyama BH, Weissman JS. The physical basis of how prion conformations determine strain phenotypes. Nature 2006; 442:585 - 9; http://dx.doi.org/10.1038/nature04922; PMID: 16810177
   PubMed Web of Science ®Google Scholar
• Immel F, Jiang Y, Wang YQ, Marchal C, Maillet L, Perrett S, et al. In vitro analysis of SpUre2p, a prion-related protein, exemplifies the relationship between amyloid and prion. J Biol Chem 2007; 282:7912 - 20; http://dx.doi.org/10.1074/jbc.M608652200; PMID: 17234629
   PubMed Web of Science ®Google Scholar
• Legname G, Nguyen HO, Peretz D, Cohen FE, DeArmond SJ, Prusiner SB. Continuum of prion protein structures enciphers a multitude of prion isolate-specified phenotypes. Proc Natl Acad Sci U S A 2006; 103:19105 - 10; http://dx.doi.org/10.1073/pnas.0608970103; PMID: 17142317
   PubMed Web of Science ®Google Scholar
• Zampieri M, Legname G, Altafini C. Investigating the conformational stability of prion strains through a kinetic replication model. PLoS Comput Biol 2009; 5:e1000420; http://dx.doi.org/10.1371/journal.pcbi.1000420; PMID: 19578427
   PubMed Web of Science ®Google Scholar
• Ayers JI, Schutt CR, Shikiya RA, Aguzzi A, Kincaid AE, Bartz JC. The strain-encoded relationship between PrP replication, stability and processing in neurons is predictive of the incubation period of disease. PLoS Pathog 2011; 7:e1001317; http://dx.doi.org/10.1371/journal.ppat.1001317; PMID: 21437239
   PubMed Web of Science ®Google Scholar
• Bett C, Joshi-Barr S, Lucero M, Trejo M, Liberski P, Kelly JW, et al. Biochemical properties of highly neuroinvasive prion strains. PLoS Pathog 2012; 8:e1002522; http://dx.doi.org/10.1371/journal.ppat.1002522; PMID: 22319450
   PubMed Web of Science ®Google Scholar
• Silveira JR, Raymond GJ, Hughson AG, Race RE, Sim VL, Hayes SF, et al. The most infectious prion protein particles. Nature 2005; 437:257 - 61; http://dx.doi.org/10.1038/nature03989; PMID: 16148934
   PubMed Web of Science ®Google Scholar
• Li J, Browning S, Mahal SP, Oelschlegel AM, Weissmann C. Darwinian evolution of prions in cell culture. Science 2010; 327:869 - 72; http://dx.doi.org/10.1126/science.1183218; PMID: 20044542
   PubMed Web of Science ®Google Scholar
• Bruce ME, Dickinson AG. Biological evidence that scrapie agent has an independent genome. J Gen Virol 1987; 68:79 - 89; http://dx.doi.org/10.1099/0022-1317-68-1-79; PMID: 3100717
   PubMed Web of Science ®Google Scholar
• Bartz JC, Marsh RF, McKenzie DI, Aiken JM. The host range of chronic wasting disease is altered on passage in ferrets. Virology 1998; 251:297 - 301; http://dx.doi.org/10.1006/viro.1998.9427; PMID: 9837794
   PubMed Web of Science ®Google Scholar
• Scheibel T, Lindquist SL. The role of conformational flexibility in prion propagation and maintenance for Sup35p. Nat Struct Biol 2001; 8:958 - 62; http://dx.doi.org/10.1038/nsb1101-958; PMID: 11685242
   PubMedGoogle Scholar
• Serio TR, Cashikar AG, Kowal AS, Sawicki GJ, Moslehi JJ, Serpell L, et al. Nucleated conformational conversion and the replication of conformational information by a prion determinant. Science 2000; 289:1317 - 21; http://dx.doi.org/10.1126/science.289.5483.1317; PMID: 10958771
   PubMed Web of Science ®Google Scholar
• Bessen RA, Marsh RF. Distinct PrP properties suggest the molecular basis of strain variation in transmissible mink encephalopathy. J Virol 1994; 68:7859 - 68; PMID: 7966576
   PubMed Web of Science ®Google Scholar
• Sigurdson CJ, Manco G, Schwarz P, Liberski P, Hoover EA, Hornemann S, et al. Strain fidelity of chronic wasting disease upon murine adaptation. J Virol 2006; 80:12303 - 11; http://dx.doi.org/10.1128/JVI.01120-06; PMID: 17020952
   PubMed Web of Science ®Google Scholar
• Sigurdson CJ, Nilsson KP, Hornemann S, Manco G, Polymenidou M, Schwarz P, et al. Prion strain discrimination using luminescent conjugated polymers. Nat Methods 2007; 4:1023 - 30; http://dx.doi.org/10.1038/nmeth1131; PMID: 18026110
   PubMed Web of Science ®Google Scholar
• Nilsson MR. Techniques to study amyloid fibril formation in vitro. Methods 2004; 34:151 - 60; http://dx.doi.org/10.1016/j.ymeth.2004.03.012; PMID: 15283924
   PubMed Web of Science ®Google Scholar
• Kimberlin RH, Walker CA. Evidence that the transmission of one source of scrapie agent to hamsters involves separation of agent strains from a mixture. J Gen Virol 1978; 39:487 - 96; http://dx.doi.org/10.1099/0022-1317-39-3-487; PMID: 96212
   PubMed Web of Science ®Google Scholar
• Polymenidou M, Stoeck K, Glatzel M, Vey M, Bellon A, Aguzzi A. Coexistence of multiple PrPSc types in individuals with Creutzfeldt-Jakob disease. Lancet Neurol 2005; 4:805 - 14; http://dx.doi.org/10.1016/S1474-4422(05)70225-8; PMID: 16297838
   PubMed Web of Science ®Google Scholar
• Taylor DM, Fernie K, McConnell I, Steele PJ. Observations on thermostable subpopulations of the unconventional agents that cause transmissible degenerative encephalopathies. Vet Microbiol 1998; 64:33 - 8; http://dx.doi.org/10.1016/S0378-1135(98)00257-0; PMID: 9874101
   PubMed Web of Science ®Google Scholar
• Safar JG, DeArmond SJ, Kociuba K, Deering C, Didorenko S, Bouzamondo-Bernstein E, et al. Prion clearance in bigenic mice. J Gen Virol 2005; 86:2913 - 23; http://dx.doi.org/10.1099/vir.0.80947-0; PMID: 16186247
   PubMed Web of Science ®Google Scholar
• Wong E, Cuervo AM. Integration of clearance mechanisms: the proteasome and autophagy. Cold Spring Harb Perspect Biol 2010; 2:a006734; http://dx.doi.org/10.1101/cshperspect.a006734; PMID: 21068151
   PubMed Web of Science ®Google Scholar
• Paar C, Wurm S, Pfarr W, Sonnleitner A, Wechselberger C. Prion protein resides in membrane microclusters of the immunological synapse during lymphocyte activation. Eur J Cell Biol 2007; 86:253 - 64; http://dx.doi.org/10.1016/j.ejcb.2007.03.001; PMID: 17449139
   PubMed Web of Science ®Google Scholar
• Mannini B, Cascella R, Zampagni M, van Waarde-Verhagen M, Meehan S, Roodveldt C, et al. Molecular mechanisms used by chaperones to reduce the toxicity of aberrant protein oligomers. Proc Natl Acad Sci U S A 2012; 109:12479 - 84; http://dx.doi.org/10.1073/pnas.1117799109; PMID: 22802614
   PubMed Web of Science ®Google Scholar
• Weissmann C, Li J, Mahal SP, Browning S. Prions on the move. EMBO Rep 2011; 12:1109 - 17; http://dx.doi.org/10.1038/embor.2011.192; PMID: 21997298
   PubMed Web of Science ®Google Scholar
• Bruce ME, Boyle A, Cousens S, McConnell I, Foster J, Goldmann W, et al. Strain characterization of natural sheep scrapie and comparison with BSE. J Gen Virol 2002; 83:695 - 704; PMID: 11842264
   PubMed Web of Science ®Google Scholar
• Bruce M, Chree A, McConnell I, Foster J, Pearson G, Fraser H. Transmission of bovine spongiform encephalopathy and scrapie to mice: strain variation and the species barrier. Philos Trans R Soc Lond B Biol Sci 1994; 343:405 - 11; http://dx.doi.org/10.1098/rstb.1994.0036; PMID: 7913758
   PubMed Web of Science ®Google Scholar
• Hill AF, Collinge J. Prion strains and species barriers. Contrib Microbiol 2004; 11:33 - 49; http://dx.doi.org/10.1159/000077061; PMID: 15077403
   PubMedGoogle Scholar
• Collinge J, Clarke AR. A general model of prion strains and their pathogenicity. Science 2007; 318:930 - 6; http://dx.doi.org/10.1126/science.1138718; PMID: 17991853
   PubMed Web of Science ®Google Scholar
• Bartz JC, Bessen RA, McKenzie D, Marsh RF, Aiken JM. Adaptation and selection of prion protein strain conformations following interspecies transmission of transmissible mink encephalopathy. J Virol 2000; 74:5542 - 7; http://dx.doi.org/10.1128/JVI.74.12.5542-5547.2000; PMID: 10823860
   PubMed Web of Science ®Google Scholar
• Falsig J, Nilsson KP, Knowles TP, Aguzzi A. Chemical and biophysical insights into the propagation of prion strains. HFSP J 2008; 2:332 - 41; http://dx.doi.org/10.2976/1.2990786; PMID: 19436493
   PubMed Web of Science ®Google Scholar
• Asante EA, Linehan JM, Desbruslais M, Joiner S, Gowland I, Wood AL, 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 2002; 21:6358 - 66; http://dx.doi.org/10.1093/emboj/cdf653; PMID: 12456643
   PubMed Web of Science ®Google Scholar
• Lloyd SE, Linehan JM, Desbruslais M, Joiner S, Buckell J, Brandner S, et al. Characterization of two distinct prion strains derived from bovine spongiform encephalopathy transmissions to inbred mice. J Gen Virol 2004; 85:2471 - 8; http://dx.doi.org/10.1099/vir.0.79889-0; PMID: 15269389
   PubMed Web of Science ®Google Scholar
• Geoghegan JC, Valdes PA, Orem NR, Deleault NR, Williamson RA, Harris BT, et al. Selective incorporation of polyanionic molecules into hamster prions. J Biol Chem 2007; 282:36341 - 53; http://dx.doi.org/10.1074/jbc.M704447200; PMID: 17940287
   PubMed Web of Science ®Google Scholar
• Deleault NR, Kascsak R, Geoghegan JC, Supattapone S. Species-dependent differences in cofactor utilization for formation of the protease-resistant prion protein in vitro. Biochemistry 2010; 49:3928 - 34; http://dx.doi.org/10.1021/bi100370b; PMID: 20377181
   PubMed Web of Science ®Google Scholar
• Deleault NR, Piro JR, Walsh DJ, Wang F, Ma J, Geoghegan JC, et al. Isolation of phosphatidylethanolamine as a solitary cofactor for prion formation in the absence of nucleic acids. Proc Natl Acad Sci U S A 2012; 109:8546 - 51; http://dx.doi.org/10.1073/pnas.1204498109; PMID: 22586108
   PubMed Web of Science ®Google Scholar
• Parsell DA, Lindquist S. The function of heat-shock proteins in stress tolerance: degradation and reactivation of damaged proteins. Annu Rev Genet 1993; 27:437 - 96; http://dx.doi.org/10.1146/annurev.ge.27.120193.002253; PMID: 8122909
   PubMed Web of Science ®Google Scholar
• Saborio GP, Permanne B, Soto C. Sensitive detection of pathological prion protein by cyclic amplification of protein misfolding. Nature 2001; 411:810 - 3; http://dx.doi.org/10.1038/35081095; PMID: 11459061
   PubMed Web of Science ®Google Scholar
• Mahal SP, Baker CA, Demczyk CA, Smith EW, Julius C, Weissmann C. Prion strain discrimination in cell culture: the cell panel assay. Proc Natl Acad Sci U S A 2007; 104:20908 - 13; http://dx.doi.org/10.1073/pnas.0710054104; PMID: 18077360
   PubMed Web of Science ®Google Scholar
• Tremblay P, Ball HL, Kaneko K, Groth D, Hegde RS, Cohen FE, et al. Mutant PrPSc conformers induced by a synthetic peptide and several prion strains. J Virol 2004; 78:2088 - 99; http://dx.doi.org/10.1128/JVI.78.4.2088-2099.2004; PMID: 14747574
   PubMed Web of Science ®Google Scholar
• Aguzzi A, Sigurdson CJ. Antiprion immunotherapy: to suppress or to stimulate?. Nat Rev Immunol 2004; 4:725 - 36; http://dx.doi.org/10.1038/nri1437; PMID: 15343371
   PubMed Web of Science ®Google Scholar
• Hilton DA, Fathers E, Edwards P, Ironside JW, Zajicek J. Prion immunoreactivity in appendix before clinical onset of variant Creutzfeldt-Jakob disease. Lancet 1998; 352:703 - 4; http://dx.doi.org/10.1016/S0140-6736(98)24035-9; PMID: 9728989
   PubMed Web of Science ®Google Scholar
• Wadsworth JD, Joiner S, Hill AF, Campbell TA, Desbruslais M, Luthert PJ, et al. Tissue distribution of protease resistant prion protein in variant Creutzfeldt-Jakob disease using a highly sensitive immunoblotting assay. Lancet 2001; 358:171 - 80; http://dx.doi.org/10.1016/S0140-6736(01)05403-4; PMID: 11476832
   PubMed Web of Science ®Google Scholar
• Mahal SP, Browning S, Li J, Suponitsky-Kroyter I, Weissmann C. Transfer of a prion strain to different hosts leads to emergence of strain variants. Proc Natl Acad Sci U S A 2010; 107:22653 - 8; http://dx.doi.org/10.1073/pnas.1013014108; PMID: 21156827
   PubMed Web of Science ®Google Scholar
• Parchi P, Giese A, Capellari S, Brown P, Schulz-Schaeffer W, Windl O, et al. Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann Neurol 1999; 46:224 - 33; http://dx.doi.org/10.1002/1531-8249(199908)46:2<224::AID-ANA12>3.0.CO;2-W; PMID: 10443888
   PubMed Web of Science ®Google Scholar
• Gambetti P, Kong Q, Zou W, Parchi P, Chen SG. Sporadic and familial CJD: classification and characterisation. Br Med Bull 2003; 66:213 - 39; http://dx.doi.org/10.1093/bmb/66.1.213; PMID: 14522861
   PubMed Web of Science ®Google Scholar
• Faucheux BA, Morain E, Diouron V, Brandel JP, Salomon D, Sazdovitch V, et al. Quantification of surviving cerebellar granule neurones and abnormal prion protein (PrPSc) deposition in sporadic Creutzfeldt-Jakob disease supports a pathogenic role for small PrPSc deposits common to the various molecular subtypes. Neuropathol Appl Neurobiol 2011; 37:500 - 12; http://dx.doi.org/10.1111/j.1365-2990.2011.01179.x; PMID: 21450052
   PubMed Web of Science ®Google Scholar
• Schoch G, Seeger H, Bogousslavsky J, Tolnay M, Janzer RC, Aguzzi A, et al. Analysis of prion strains by PrPSc profiling in sporadic Creutzfeldt-Jakob disease. PLoS Med 2006; 3:e14; http://dx.doi.org/10.1371/journal.pmed.0030014; PMID: 16354106
   PubMed Web of Science ®Google Scholar
• Puoti G, Giaccone G, Rossi G, Canciani B, Bugiani O, Tagliavini F. Sporadic Creutzfeldt-Jakob disease: co-occurrence of different types of PrP(Sc) in the same brain. Neurology 1999; 53:2173 - 6; http://dx.doi.org/10.1212/WNL.53.9.2173; PMID: 10599800
   PubMed Web of Science ®Google Scholar
• Cali I, Castellani R, Alshekhlee A, Cohen Y, Blevins J, Yuan J, et al. Co-existence of scrapie prion protein types 1 and 2 in sporadic Creutzfeldt-Jakob disease: its effect on the phenotype and prion-type characteristics. Brain 2009; 132:2643 - 58; http://dx.doi.org/10.1093/brain/awp196; PMID: 19734292
   PubMed Web of Science ®Google Scholar
• Nonno R, Di Bari MA, Cardone F, Vaccari G, Fazzi P, Dell’Omo G, et al. Efficient transmission and characterization of Creutzfeldt-Jakob disease strains in bank voles. PLoS Pathog 2006; 2:e12; http://dx.doi.org/10.1371/journal.ppat.0020012; PMID: 16518470
   PubMed Web of Science ®Google Scholar
• Bishop MT, Will RG, Manson JC. Defining sporadic Creutzfeldt-Jakob disease strains and their transmission properties. Proc Natl Acad Sci U S A 2010; 107:12005 - 10; http://dx.doi.org/10.1073/pnas.1004688107; PMID: 20547859
   PubMed Web of Science ®Google Scholar
• Yull HM, Ritchie DL, Langeveld JP, van Zijderveld FG, Bruce ME, Ironside JW, et al. Detection of type 1 prion protein in variant Creutzfeldt-Jakob disease. Am J Pathol 2006; 168:151 - 7; http://dx.doi.org/10.2353/ajpath.2006.050766; PMID: 16400018
   PubMed Web of Science ®Google Scholar
• Hill AF, Desbruslais M, Joiner S, Sidle KC, Gowland I, Collinge J, et al. The same prion strain causes vCJD and BSE. Nature 1997; 389:448 - 50, 526; http://dx.doi.org/10.1038/38925; PMID: 9333232
   PubMed Web of Science ®Google Scholar
• Brandel JP, Heath CA, Head MW, Levavasseur E, Knight R, Laplanche JL, et al. Variant Creutzfeldt-Jakob disease in France and the United Kingdom: Evidence for the same agent strain. Ann Neurol 2009; 65:249 - 56; http://dx.doi.org/10.1002/ana.21583; PMID: 19334063
   PubMed Web of Science ®Google Scholar
• Minor P, Newham J, Jones N, Bergeron C, Gregori L, Asher D, et al, WHO Working Group on International Reference Materials for the Diagnosis and Study of Transmissible Spongiform Encephalopathies. Standards for the assay of Creutzfeldt-Jakob disease specimens. J Gen Virol 2004; 85:1777 - 84; http://dx.doi.org/10.1099/vir.0.79959-0; PMID: 15166463
   PubMed Web of Science ®Google Scholar
• Béringue V, Herzog L, Reine F, Le Dur A, Casalone C, Vilotte JL, et al. Transmission of atypical bovine prions to mice transgenic for human prion protein. Emerg Infect Dis 2008; 14:1898 - 901; http://dx.doi.org/10.3201/eid1412.080941; PMID: 19046515
   PubMed Web of Science ®Google Scholar
• Béringue V, Le Dur A, Tixador P, Reine F, Lepourry L, Perret-Liaudet A, et al. Prominent and persistent extraneural infection in human PrP transgenic mice infected with variant CJD. PLoS One 2008; 3:e1419; http://dx.doi.org/10.1371/journal.pone.0001419; PMID: 18183299
   PubMedGoogle Scholar
• Ross CA, Poirier MA. Protein aggregation and neurodegenerative disease. Nat Med 2004; 10:Suppl S10 - 7; http://dx.doi.org/10.1038/nm1066; PMID: 15272267
   PubMedGoogle Scholar
• Aguzzi A, Calella AM. Prions: protein aggregation and infectious diseases. Physiol Rev 2009; 89:1105 - 52; http://dx.doi.org/10.1152/physrev.00006.2009; PMID: 19789378
   PubMed Web of Science ®Google Scholar