Eight prion strains have PrPSc molecules with different conformations (original) (raw)

• Prusiner, S.B. Prion diseases and the BSE crisis. Science 278, 245–251 (1997).
  Article CAS PubMed Google Scholar
• Pattison, I.H. & Millson, G.C. Scrapie produced experimentally in goats with special reference to the clinical syndrome. J. Comp. Pathol. 71, 101–108 ( 1961).
  Article CAS PubMed Google Scholar
• Sigurdsson, B. Rida, a chronic encephalitis of sheep with general remarks on infections which develop slowly and some of their special characteristics. Br. Vet. J. 110, 341–354 ( 1954).
  Article Google Scholar
• Gajdusek, D.C. Unconventional viruses and the origin and disappearance of kuru. Science 197, 943–960 ( 1977).
  Article CAS PubMed Google Scholar
• Bruce, M.E. & Dickinson, A.G. Biological evidence that the scrapie agent has an independent genome. J. Gen. Virol. 68, 79–89 (1987).
  Article PubMed Google Scholar
• Dickinson, A.G. & Outram, G.W. in Novel Infectious Agents and the Central Nervous System. Ciba Foundation Symposium 135 (eds. Bock, G. & Marsh, J.) 63–83 (John Wiley and Sons, Chichester, England, 1988).
  Google Scholar
• Bruce, M.E. & Fraser, H. Scrapie strain variation and its implications. Curr. Top. Microbiol. Immunol. 172, 125–138 (1991).
  CAS PubMed Google Scholar
• Fraser, H. & Dickinson, A.G. The sequential development of the brain lesions of scrapie in three strains of mice. J. Comp. Pathol. 78, 301–311 ( 1968).
  Article CAS PubMed Google Scholar
• Bruce, M.E., McBride, P.A. & Farquhar, C.F. Precise targeting of the pathology of the sialoglycoprotein, PrP, and vacuolar degeneration in mouse scrapie. Neurosci. Lett. 102, 1–6 (1989 ).
  Article CAS PubMed Google Scholar
• Taraboulos, A. et al. Regional mapping of prion proteins in brains. Proc. Natl. Acad. Sci. USA 89, 7620– 7624 (1992).
  Article CAS PubMed PubMed Central Google Scholar
• Scott, M.R. et al. Propagation of prion strains through specific conformers of the prion protein. J. Virol. 71, 9032 –9044 (1997).
  CAS PubMed PubMed Central Google Scholar
• Prusiner, S.B. Molecular biology of prion diseases. Science 252, 1515–1522 (1991).
  Article CAS PubMed Google Scholar
• Cohen, F.E. et al. Structural clues to prion replication. Science 264, 530–531 ( 1994).
  Article CAS PubMed Google Scholar
• Bessen, R.A. & Marsh, R.F. Distinct PrP properties suggest the molecular basis of strain variation in transmissible mink encephalopathy. J. Virol. 68, 7859–7868 (1994).
  CAS PubMed PubMed Central Google Scholar
• Telling, G.C. et al. Evidence for the conformation of the pathologic isoform of the prion protein enciphering and propagating prion diversity. Science 274, 2079–2082 ( 1996).
  Article CAS PubMed Google Scholar
• Stahl, N. et al. Structural analysis of the scrapie prion protein using mass spectrometry and amino acid sequencing. Biochemistry 32, 1991–2002 (1993).
  Article CAS PubMed Google Scholar
• Serban, D., Taraboulos, A., DeArmond, S.J. & Prusiner, S.B. Rapid detection of Creutzfeldt-Jakob disease and scrapie prion proteins. Neurology 40, 110–117 ( 1990).
  Article CAS PubMed Google Scholar
• Peretz, D. et al. A conformational transition at the N terminus of the prion protein features in formation of the scrapie isoform. J. Mol. Biol. 273, 614–622 ( 1997).
  Article CAS PubMed Google Scholar
• Mehlhorn, I. et al. High-level expression and characterization of a purified 142-residue polypeptide of the prion protein. Biochemistry 35, 5528–5537 (1996).
  Article CAS PubMed Google Scholar
• Kascsak, R.J. et al. Mouse polyclonal and monoclonal antibody to scrapie-associated fibril proteins. J. Virol. 61, 3688– 3693 (1987).
  CAS PubMed PubMed Central Google Scholar
• Hemmilä, I. & Harju, R. Time-resolved fluorometry . in Bioanalytical Applications of Labelling Technologies (eds. Hemmilä, I., Ståhlberg, T. & Mottram, P.) 113– 119 (Wallac Oy, Turku, Finland, 1995).
  Google Scholar
• Büeler, H. et al. Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein. Nature 356, 577–582 (1992).
  Article PubMed Google Scholar
• Bessen, R.A. & Marsh, R.F. Biochemical and physical properties of the prion protein from two strains of the transmissible mink encephalopathy agent. J. Virol. 66, 2096– 2101 (1992).
  CAS PubMed PubMed Central Google Scholar
• Prusiner, S.B., Scott, M.R., DeArmond, S.J. & Cohen, F.E. Prion protein biology. Cell 93, 337– 348 (1998).
  Article CAS PubMed Google Scholar
• Safar, J., Roller, P.P., Gajdusek, D.C. & Gibbs, C.J., Jr. Conformational transitions, dissociation, and unfolding of scrapie amyloid (prion) protein. J. Biol. Chem. 268, 20276 –20284 (1993).
  CAS PubMed Google Scholar
• Prusiner, S.B. et al. Scrapie prions aggregate to form amyloid-like birefringent rods. Cell 35, 349–358 (1983).
  Article CAS PubMed Google Scholar
• Prusiner, S.B. et al. Measurement of the scrapie agent using an incubation time interval assay. Ann. Neurol. 11, 353 –358 (1982).
  Article CAS PubMed Google Scholar
• James, T.L. et al. Solution structure of a 142-residue recombinant prion protein corresponding to the infectious fragment of the scrapie isoform. Proc. Natl. Acad. Sci. USA 94, 10086– 10091 (1997).
  Article CAS PubMed PubMed Central Google Scholar
• Pan, K.-M. et al. Conversion of α-helices into ß-sheets features in the formation of the scrapie prion proteins. Proc. Natl. Acad. Sci. USA 90, 10962–10966 (1993).
  Article CAS PubMed PubMed Central Google Scholar
• Pergami, P., Jaffe, H. & Safar, J. Semipreparative chromatographic method to purify the normal cellular isoform of the prion protein in nondenatured form. Anal. Biochem. 236, 63–73 ( 1996).
  Article CAS PubMed Google Scholar
• Williamson, R.A. et al. Circumventing tolerance to generate autologous monoclonal antibodies to the prion protein. Proc. Natl. Acad. Sci. USA 93, 7279–7282 (1996).
  Article CAS PubMed PubMed Central Google Scholar
• Huang, Z., Prusiner, S.B. & Cohen, F.E. Scrapie prions: a three-dimensional model of an infectious fragment. Folding & Design 1, 13– 19 (1995).
  Article CAS Google Scholar
• Meyer, N. et al. Search for a putative scrapie genome in purified prion fractions reveals a paucity of nucleic acids. J. Gen. Virol. 72, 37–49 (1991).
  Article CAS PubMed Google Scholar
• Kellings, K., Prusiner, S.B. & Riesner, D. Nucleic acids in prion preparations: unspecific background or essential component? Phil. Trans. R. Soc. Lond. B 343, 425–430 (1994).
  Article CAS Google Scholar
• Kellings, K., Meyer, N., Mirenda, C., Prusiner, S.B. & Riesner, D. Further analysis of nucleic acids in purified scrapie prion preparations by improved return refocussing gel electrophoresis (RRGE). J. Gen. Virol. 73, 1025– 1029 (1992).
  Article CAS PubMed Google Scholar
• Prusiner, S.B. Prions. Les Prix Nobel (in the press).
• Anfinsen, C.B. Principles that govern the folding of protein chains. Science 181, 223–230 (1973).
  Article CAS PubMed Google Scholar
• Kaneko, K. et al. Evidence for protein X binding to a discontinuous epitope on the cellular prion protein during scrapie prion propagation. Proc. Natl. Acad. Sci. USA 94, 10069– 10074 (1997).
  Article CAS PubMed PubMed Central Google Scholar
• Cohen, F.E. & Prusiner, S.B. Pathologic conformations of prion proteins. Annu. Rev. Biochem. 67, 793– 819 (1998).
  Article CAS PubMed Google Scholar
• Collinge, J., Sidle, K.C.L., Meads, J., Ironside, J. & Hill, A.F. Molecular analysis of prion strain variation and the aetiology of "new variant" CJD. Nature 383, 685–690 (1996).
  Article CAS PubMed Google Scholar
• Endo, T., Groth, D., Prusiner, S.B. & Kobata, A. Diversity of oligosaccharide structures linked to asparagines of the scrapie prion protein. Biochemistry 28, 8380– 8388 (1989).
  Article CAS PubMed Google Scholar
• Borchelt, D.R., Scott, M., Taraboulos, A., Stahl, N. & Prusiner, S.B. Scrapie and cellular prion proteins differ in their kinetics of synthesis and topology in cultured cells. J. Cell Biol. 110, 743–752 (1990).
  Article CAS PubMed Google Scholar
• Caughey, B. & Raymond, G.J. The scrapie-associated form of PrP is made from a cell surface precursor that is both protease- and phospholipase-sensitive. J. Biol. Chem. 266, 18217– 18223 (1991).
  CAS PubMed Google Scholar
• Taraboulos, A. et al. Acquisition of protease resistance by prion proteins in scrapie-infected cells does not require asparagine-linked glycosylation. Proc. Natl. Acad. Sci. USA 87, 8262– 8266 (1990).
  Article CAS PubMed PubMed Central Google Scholar
• DeArmond, S.J. et al. Selective neuronal targeting in prion disease. Neuron 19, 1337–1348 ( 1997).
  Article CAS PubMed Google Scholar
• Will, R.G. et al. A new variant of Creutzfeldt-Jakob disease in the UK. Lancet 347, 921–925 ( 1996).
  Article CAS PubMed Google Scholar
• Brown, P. in Transmissible Subacute Spongiform Encephalopathies: Prion Diseases (eds. Court, L. & Dodet, B.) 447–450 (Elsevier, Paris, 1996).
  Google Scholar
• Prusiner, S.B. et al. Further purification and characterization of scrapie prions. Biochemistry 21, 6942– 6950 (1982).
  Article CAS PubMed Google Scholar
• Telling, G.C. et al. Transmission of Creutzfeldt-Jakob disease from humans to transgenic mice expressing chimeric human-mouse prion protein. Proc. Natl. Acad. Sci. USA 91, 9936– 9940 (1994).
  Article CAS PubMed PubMed Central Google Scholar
• Scott, M.R. et al. Identification of a prion protein epitope modulating transmission of bovine spongiform encephalopathy prions to transgenic mice. Proc. Natl. Acad. Sci. USA 94, 14279– 14284 (1997).
  Article CAS PubMed PubMed Central Google Scholar
• Marsh, R.F. & Kimberlin, R.H. Comparison of scrapie and transmissible mink encephalopathy in hamsters. II. Clinical signs, pathology and pathogenesis. J. Infect. Dis. 131, 104– 110 (1975).
  Article CAS PubMed Google Scholar
• Kimberlin, R. & Walker, C. Characteristics of a short incubation model of scrapie in the golden hamster. J. Gen. Virol. 34, 295–304 (1977).
  Article CAS PubMed Google Scholar
• Gibbs, C.J., Jr., Safar, J., Sulima, M.P., Bacote, A.E. & San Martin, R.A. in Bovine Spongiform Encephalopathy: The BSE Dilemma (ed. Gibbs, C.J., Jr.) 84–91 (Springer, New York, 1996).
  Book Google Scholar
• Marsh, R.F., Bessen, R.A., Lehmann, S. & Hartsough, G.R. Epidemiological and experimental studies on a new incident of transmissible mink encephalopathy. J. Gen. Virol. 72, 589–594 (1991).
  Article PubMed Google Scholar
• Dickinson, A.G. in Slow Virus Diseases of Animals and Man (ed. Kimberlin, R.H.) 209–241 (North-Holland Publishing, Amsterdam, 1976).
  Google Scholar
• Kimberlin, R.H., Cole, S. & Walker, C.A. Pathogenesis of scrapie is faster when infection is intraspinal instead of intracerebral. Microb. Pathog. 2, 405–415 (1987).
  Article CAS PubMed Google Scholar
• Turk, E., Teplow, D.B., Hood, L.E. & Prusiner, S.B. Purification and properties of the cellular and scrapie hamster prion proteins. Eur. J. Biochem. 176, 21–30 (1988).
  Article CAS PubMed Google Scholar
• Safar, J., Roller, P.P., Gajdusek, D.C. & Gibbs, C.J., Jr. Scrapie amyloid (prion) protein has the conformational characteristics of an aggregated molten globule folding intermediate. Biochemistry 33, 8375–8383 ( 1994).
  Article CAS PubMed Google Scholar