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Science 9 April 1982:
Vol. 216. no. 4542, pp. 136 - 144
DOI: 10.1126/science.6801762

Articles

Science, Vol 216, Issue 4542, 136-144
Copyright © 1982 by American Association for the Advancement of Science


articles

Novel proteinaceous infectious particles cause scrapie

SB Prusiner

After infection and a prolonged incubation period, the scrapie agent causes a degenerative disease of the central nervous system in sheep and goats. Six lines of evidence including sensitivity to proteases demonstrate that this agent contains a protein that is required for infectivity. Although the scrapie agent is irreversibly inactivated by alkali, five procedures with more specificity for modifying nucleic acids failed to cause inactivation. The agent shows heterogeneity with respect to size, apparently a result of its hydrophobicity; the smallest form may have a molecular weight of 50,000 or less. Because the novel properties of the scrapie agent distinguish it from viruses, plasmids, and viroids, a new term "prion" is proposed to denote a small proteinaceous infectious particle which is resistant to inactivation by most procedures that modify nucleic acids. Knowledge of the scrapie agent structure may have significance for understanding the causes of several degenerative diseases.


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A Novel Protective Prion Protein Variant that Colocalizes with Kuru Exposure.
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Prions: Protein Aggregation and Infectious Diseases.
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Protective effect of the T112 PrP variant in sheep challenged with bovine spongiform encephalopathy.
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Distinct Structures of Scrapie Prion Protein (PrPSc)-seeded Versus Spontaneous Recombinant Prion Protein Fibrils Revealed by Hydrogen/Deuterium Exchange.
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Measuring prions by bioluminescence imaging.
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PNAS 106, 15002-15006
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Conformational Properties of {beta}-PrP.
L. L. P. Hosszu, C. R. Trevitt, S. Jones, M. Batchelor, D. J. Scott, G. S. Jackson, J. Collinge, J. P. Waltho, and A. R. Clarke (2009)
J. Biol. Chem. 284, 21981-21990
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Immunological mimicry of PrPC-PrPSc interactions: antibody-induced PrP misfolding.
L. Li, W. Guest, A. Huang, S. S. Plotkin, and N. R. Cashman (2009)
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New Approach for M-Cell-Specific Molecules Screening by Comprehensive Transcriptome Analysis.
G. Nakato, S. Fukuda, K. Hase, R. Goitsuka, M. D. Cooper, and H. Ohno (2009)
DNA Res 16, 227-235
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Alternative Translation Initiation Generates Cytoplasmic Sheep Prion Protein.
C. Lund, C. M. Olsen, S. Skogtvedt, H. Tveit, K. Prydz, and M. A. Tranulis (2009)
J. Biol. Chem. 284, 19668-19678
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Phosphorylation of Prion Protein at Serine 43 Induces Prion Protein Conformational Change.
P. N. Giannopoulos, C. Robertson, J. Jodoin, H. Paudel, S. A. Booth, and A. C. LeBlanc (2009)
J. Neurosci. 29, 8743-8751
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Prion Protein Glycosylation Is Not Required for Strain-Specific Neurotropism.
J. R. Piro, B. T. Harris, K. Nishina, C. Soto, R. Morales, J. R. Rees, and S. Supattapone (2009)
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Detection of classical and atypical/Nor98 scrapie by the paraffin-embedded tissue blot method.
W. M. Wemheuer, S. L. Benestad, A. Wrede, W. E. Wemheuer, B. Brenig, B. Bratberg, and W. J. Schulz-Schaeffer (2009)
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Heterologous Stacking of Prion Protein Peptides Reveals Structural Details of Fibrils and Facilitates Complete Inhibition of Fibril Growth.
R. S. Boshuizen, V. Schulz, M. Morbin, G. Mazzoleni, R. H. Meloen, and J. P. M. Langedijk (2009)
J. Biol. Chem. 284, 12809-12820
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Selfish prion of Rnq1 mutant in yeast.
H. Kurahashi, S. Shibata, M. Ishiwata, and Y. Nakamura (2009)
Genes Cells 14, 659-668
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Olfactory System Involvement in Natural Scrapie Disease.
C. Corona, C. Porcario, F. Martucci, B. Iulini, B. Manea, M. Gallo, C. Palmitessa, C. Maurella, M. Mazza, M. Pezzolato, et al. (2009)
J. Virol. 83, 3657-3667
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Identification of Adjacent Binding Sites for the YY1 and E4BP4 Transcription Factors in the Ovine PrP (Prion) Gene Promoter.
S. T. G. Burgess, C. Shen, L. A. Ferguson, G. T. O'Neill, K. Docherty, N. Hunter, and W. Goldmann (2009)
J. Biol. Chem. 284, 6716-6724
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Highly sensitive, quantitative cell-based assay for prions adsorbed to solid surfaces.
J. A. Edgeworth, G. S. Jackson, A. R. Clarke, C. Weissmann, and J. Collinge (2009)
PNAS 106, 3479-3483
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Three serial passages of bovine spongiform encephalopathy in sheep do not significantly affect discriminatory test results.
M. Stack, L. Gonzalez, M. Jeffrey, S. Martin, C. Macaldowie, M. Chaplin, J. Thorne, R. Sayers, L. Davis, J. Bramwell, et al. (2009)
J. Gen. Virol. 90, 764-768
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Prion Variants and Species Barriers Among Saccharomyces Ure2 Proteins.
H. K. Edskes, L. M. McCann, A. M. Hebert, and R. B. Wickner (2009)
Genetics 181, 1159-1167
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Crystal structure of human prion protein bound to a therapeutic antibody.
S. V. Antonyuk, C. R. Trevitt, R. W. Strange, G. S. Jackson, D. Sangar, M. Batchelor, S. Cooper, C. Fraser, S. Jones, T. Georgiou, et al. (2009)
PNAS 106, 2554-2558
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The NatA Acetyltransferase Couples Sup35 Prion Complexes to the [PSI+] Phenotype.
J. A. Pezza, S. X. Langseth, R. Raupp Yamamoto, S. M. Doris, S. P. Ulin, A. R. Salomon, and T. R. Serio (2009)
Mol. Biol. Cell 20, 1068-1080
   Abstract »    Full Text »    PDF »
A G-protein {gamma} subunit mimic is a general antagonist of prion propagation in Saccharomyces cerevisiae.
M. Ishiwata, H. Kurahashi, and Y. Nakamura (2009)
PNAS 106, 791-796
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Regulation of Prion Gene Expression by Transcription Factors SP1 and Metal Transcription Factor-1.
S. A. Bellingham, L. A. Coleman, C. L. Masters, J. Camakaris, and A. F. Hill (2009)
J. Biol. Chem. 284, 1291-1301
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De novo generation of a transmissible spongiform encephalopathy by mouse transgenesis.
C. J. Sigurdson, K. P. R. Nilsson, S. Hornemann, M. Heikenwalder, G. Manco, P. Schwarz, D. Ott, T. Rulicke, P. P. Liberski, C. Julius, et al. (2009)
PNAS 106, 304-309
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Prion Strain Targeting Independent of Strain-Specific Neuronal Tropism.
J. I. Ayers, A. E. Kincaid, and J. C. Bartz (2009)
J. Virol. 83, 81-87
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Prion Protein Amyloid Formation under Native-like Conditions Involves Refolding of the C-terminal {alpha}-Helical Domain.
N. J. Cobb, A. C. Apetri, and W. K. Surewicz (2008)
J. Biol. Chem. 283, 34704-34711
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Identifying Key Components of the PrPC-PrPSc Replicative Interface.
G. C. Abalos, J. T. Cruite, A. Bellon, S. Hemmers, J. Akagi, J. A. Mastrianni, R. A. Williamson, and L. Solforosi (2008)
J. Biol. Chem. 283, 34021-34028
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Association of a null allele of SPRN with variant Creutzfeldt-Jakob disease.
J A Beck, T A Campbell, G Adamson, M Poulter, J B Uphill, E Molou, J Collinge, and S Mead (2008)
J. Med. Genet. 45, 813-817
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The changing face of kuru: a personal perspective.
J. D Mathews (2008)
Phil Trans R Soc B 363, 3679-3684
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Lessons of kuru research: background to recent studies with some personal reflections.
J. Collinge (2008)
Phil Trans R Soc B 363, 3689-3696
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The epidemiology of kuru: monitoring the epidemic from its peak to its end.
M. P Alpers (2008)
Phil Trans R Soc B 363, 3707-3713
   Abstract »    Full Text »    PDF »
A clinical study of kuru patients with long incubation periods at the end of the epidemic in Papua New Guinea.
J. Collinge, J. Whitfield, E. McKintosh, A. Frosh, S. Mead, A. F Hill, S. Brandner, D. Thomas, and M. P Alpers (2008)
Phil Trans R Soc B 363, 3725-3739
   Abstract »    Full Text »    PDF »
The origin of the prion agent of kuru: molecular and biological strain typing.
J. D.F Wadsworth, S. Joiner, J. M Linehan, E. A Asante, S. Brandner, and J. Collinge (2008)
Phil Trans R Soc B 363, 3747-3753
   Abstract »    Full Text »    PDF »
Central and peripheral pathology of kuru: pathological analysis of a recent case and comparison with other forms of human prion disease.
S. Brandner, J. Whitfield, K. Boone, A. Puwa, C. O'Malley, J. M Linehan, S. Joiner, F. Scaravilli, I. Calder, M. P. Alpers, et al. (2008)
Phil Trans R Soc B 363, 3755-3763
   Abstract »    Full Text »    PDF »
Molecular and Transmission Characteristics of Primary-Passaged Ovine Scrapie Isolates in Conventional and Ovine PrP Transgenic Mice.
A. M. Thackray, L. Hopkins, J. Spiropoulos, and R. Bujdoso (2008)
J. Virol. 82, 11197-11207
   Abstract »    Full Text »    PDF »
Small-Ruminant Lentivirus Enhances PrPSc Accumulation in Cultured Sheep Microglial Cells.
J. B. Stanton, D. P. Knowles, K. I. O'Rourke, L. M. Herrmann-Hoesing, B. A. Mathison, and T. V. Baszler (2008)
J. Virol. 82, 9839-9847
   Abstract »    Full Text »    PDF »
The effects of prion protein proteolysis and disaggregation on the strain properties of hamster scrapie.
A. M. Deleault, N. R. Deleault, B. T. Harris, J. R. Rees, and S. Supattapone (2008)
J. Gen. Virol. 89, 2642-2650
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A collaborative Canadian-United Kingdom evaluation of an immunohistochemistry protocol to diagnose bovine spongiform encephalopathy.
L. Manning, K. I. O'Rourke, D. P. Knowles, S. A. Marsh, Y. I. Spencer, E. Moffat, G. A.H. Wells, and S. Czub (2008)
J Vet Diagn Invest 20, 504-508
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A Regulatory Role of the Rnq1 Nonprion Domain for Prion Propagation and Polyglutamine Aggregates.
H. Kurahashi, M. Ishiwata, S. Shibata, and Y. Nakamura (2008)
Mol. Cell. Biol. 28, 3313-3323
   Abstract »    Full Text »    PDF »
Amyloid Fibrils of the HET-s(218-289) Prion Form a {beta} Solenoid with a Triangular Hydrophobic Core.
C. Wasmer, A. Lange, H. Van Melckebeke, A. B. Siemer, R. Riek, and B. H. Meier (2008)
Science 319, 1523-1526
   Abstract »    Full Text »    PDF »
The elk PRNP codon 132 polymorphism controls cervid and scrapie prion propagation.
K. M. Green, S. R. Browning, T. S. Seward, J. E. Jewell, D. L. Ross, M. A. Green, E. S. Williams, E. A. Hoover, and G. C. Telling (2008)
J. Gen. Virol. 89, 598-608
   Abstract »    Full Text »    PDF »
Protein Misfolding and Neurodegeneration.
C. Soto and L. D. Estrada (2008)
Arch Neurol 65, 184-189
   Abstract »    Full Text »    PDF »
Unraveling prion strains with cell biology and organic chemistry.
A. Aguzzi (2008)
PNAS 105, 11-12
   Full Text »    PDF »
Decontamination of surgical instruments from prions. II. In vivo findings with a model system for testing the removal of scrapie infectivity from steel surfaces.
K. Lemmer, M. Mielke, C. Kratzel, M. Joncic, M. Oezel, G. Pauli, and M. Beekes (2008)
J. Gen. Virol. 89, 348-358
   Abstract »    Full Text »    PDF »
Selective Incorporation of Polyanionic Molecules into Hamster Prions.
J. C. Geoghegan, P. A. Valdes, N. R. Orem, N. R. Deleault, R. A. Williamson, B. T. Harris, and S. Supattapone (2007)
J. Biol. Chem. 282, 36341-36353
   Abstract »    Full Text »    PDF »
Influence of Water, Fat, and Glycerol on the Mechanism of Thermal Prion Inactivation.
H. Muller, L. Stitz, H. Wille, S. B. Prusiner, and D. Riesner (2007)
J. Biol. Chem. 282, 35855-35867
   Abstract »    Full Text »    PDF »
High Titers of Transmissible Spongiform Encephalopathy Infectivity Associated with Extremely Low Levels of PrPSc in Vivo.
R. M. Barron, S. L. Campbell, D. King, A. Bellon, K. E. Chapman, R. A. Williamson, and J. C. Manson (2007)
J. Biol. Chem. 282, 35878-35886
   Abstract »    Full Text »    PDF »
Medical Mineralogy and Geochemistry: An Interfacial Science.
N. Sahai (2007)
Elements 3, 381-384
   Abstract »    Full Text »    PDF »
Role of Hsp104 in the Propagation and Inheritance of the [Het-s] Prion.
L. Malato, S. Dos Reis, L. Benkemoun, R. Sabate, and S. J. Saupe (2007)
Mol. Biol. Cell 18, 4803-4812
   Abstract »    Full Text »    PDF »
Molecular architecture of human prion protein amyloid: A parallel, in-register -structure.
N. J. Cobb, F. D. Sonnichsen, H. Mchaourab, and W. K. Surewicz (2007)
PNAS 104, 18946-18951
   Abstract »    Full Text »    PDF »
Molecular Characterization of the Rocky Mountain Elk (Cervus elaphus nelsoni) PRNP Putative Promoter.
C. M. Seabury, C. A. Gill, J. W. Templeton, J. B. Dyar, J. N. Derr, D. L. Adelson, E. Owens, D. S. Davis, D. C. Kraemer, and J. E. Womack (2007)
J. Hered.
   Abstract »    Full Text »    PDF »
Mouse-Adapted Ovine Scrapie Prion Strains Are Characterized by Different Conformers of PrPSc.
A. M. Thackray, L. Hopkins, M. A. Klein, and R. Bujdoso (2007)
J. Virol. 81, 12119-12127
   Abstract »    Full Text »    PDF »
A General Model of Prion Strains and Their Pathogenicity.
J. Collinge and A. R. Clarke (2007)
Science 318, 930-936
   Abstract »    Full Text »    PDF »
PrPc Does Not Mediate Internalization of PrPSc but Is Required at an Early Stage for De Novo Prion Infection of Rov Cells.
S. Paquet, N. Daude, M.-P. Courageot, J. Chapuis, H. Laude, and D. Vilette (2007)
J. Virol. 81, 10786-10791
   Abstract »    Full Text »    PDF »
Polylactide-Coglycolide Microspheres CoEncapsulating Recombinant Tandem Prion Protein with CpG-Oligonucleotide Break Self-Tolerance to Prion Protein in Wild-Type Mice and Induce CD4 and CD8 T Cell Responses.
G. Kaiser-Schulz, A. Heit, L. Quintanilla-Martinez, F. Hammerschmidt, S. Hess, L. Jennen, H. Rezaei, H. Wagner, and H. M. Schatzl (2007)
J. Immunol. 179, 2797-2807
   Abstract »    Full Text »    PDF »
Species-specific Differences in the Intermediate States of Human and Syrian Hamster Prion Protein Detected by High Pressure NMR Spectroscopy.
W. Kremer, N. Kachel, K. Kuwata, K. Akasaka, and H. R. Kalbitzer (2007)
J. Biol. Chem. 282, 22689-22698
   Abstract »    Full Text »    PDF »
Hot spots in prion protein for pathogenic conversion.
K. Kuwata, N. Nishida, T. Matsumoto, Y. O. Kamatari, J. Hosokawa-Muto, K. Kodama, H. K. Nakamura, K. Kimura, M. Kawasaki, Y. Takakura, et al. (2007)
PNAS 104, 11921-11926
   Abstract »    Full Text »    PDF »
From the Cover: Formation of native prions from minimal components in vitro.
N. R. Deleault, B. T. Harris, J. R. Rees, and S. Supattapone (2007)
PNAS 104, 9741-9746
   Abstract »    Full Text »    PDF »
PrPSc in Salivary Glands of Scrapie-Affected Sheep.
M. Vascellari, R. Nonno, F. Mutinelli, M. Bigolaro, M. A. Di Bari, E. Melchiotti, S. Marcon, C. D'Agostino, G. Vaccari, M. Conte, et al. (2007)
J. Virol. 81, 4872-4876
   Abstract »    Full Text »    PDF »
Resistance to Chronic Wasting Disease in Transgenic Mice Expressing a Naturally Occurring Allelic Variant of Deer Prion Protein.
K. Meade-White, B. Race, M. Trifilo, A. Bossers, C. Favara, R. Lacasse, M. Miller, E. Williams, M. Oldstone, R. Race, et al. (2007)
J. Virol. 81, 4533-4539
   Abstract »    Full Text »    PDF »
Diversity in prion protein oligomerization pathways results from domain expansion as revealed by hydrogen/deuterium exchange and disulfide linkage.
F. Eghiaian, T. Daubenfeld, Y. Quenet, M. van Audenhaege, A.-P. Bouin, G. van der Rest, J. Grosclaude, and H. Rezaei (2007)
PNAS 104, 7414-7419
   Abstract »    Full Text »    PDF »
Human prion proteins with pathogenic mutations share common conformational changes resulting in enhanced binding to glycosaminoglycans.
S. Yin, N. Pham, S. Yu, C. Li, P. Wong, B. Chang, S.-C. Kang, E. Biasini, P. Tien, D. A. Harris, et al. (2007)
PNAS 104, 7546-7551
   Abstract »    Full Text »    PDF »
Polymorphisms of the prion protein gene coding region in born-after-the-reinforced-ban (BARB) bovine spongiform encephalopathy cattle in Great Britain.
G. C. Saunders, P. C. Griffiths, S. Cawthraw, A. C. Tout, P. Wiener, J. A. Woolliams, J. L. Williams, and O. Windl (2007)
J. Gen. Virol. 88, 1374-1378
   Abstract »    Full Text »    PDF »
Immunological differences between susceptible and resistant sheep during the preclinical phase of scrapie infection.
S. L. Eaton, M. Rocchi, L. Gonzalez, S. Hamilton, J. Finlayson, J. Sales, M. Jeffrey, P. J. Steele, M. P. Dagleish, S. M. Rodger, et al. (2007)
J. Gen. Virol. 88, 1384-1391
   Abstract »    Full Text »    PDF »
In Vitro Analysis of SpUre2p, a Prion-related Protein, Exemplifies the Relationship between Amyloid and Prion.
F. Immel, Y. Jiang, Y.-Q. Wang, C. Marchal, L. Maillet, S. Perrett, and C. Cullin (2007)
J. Biol. Chem. 282, 7912-7920
   Abstract »    Full Text »    PDF »
Oligomerization of the Human Prion Protein Proceeds via a Molten Globule Intermediate.
R. Gerber, A. Tahiri-Alaoui, P. J. Hore, and W. James (2007)
J. Biol. Chem. 282, 6300-6307
   Abstract »    Full Text »    PDF »
Dynamics and genetics of PrPSc placental accumulation in sheep.
C. Lacroux, F. Corbiere, G. Tabouret, S. Lugan, P. Costes, J. Mathey, J. M. Delmas, J. L. Weisbecker, G. Foucras, H. Cassard, et al. (2007)
J. Gen. Virol. 88, 1056-1061
   Abstract »    Full Text »    PDF »
Microglial Cell Line Established from Prion Protein-Overexpressing Mice Is Susceptible to Various Murine Prion Strains.
Y. Iwamaru, T. Takenouchi, K. Ogihara, M. Hoshino, M. Takata, M. Imamura, Y. Tagawa, H. Hayashi-Kato, Y. Ushiki-Kaku, Y. Shimizu, et al. (2007)
J. Virol. 81, 1524-1527
   Abstract »    Full Text »    PDF »
Importance of the Hsp70 ATPase Domain in Yeast Prion Propagation.
H. M. Loovers, E. Guinan, and G. W. Jones (2007)
Genetics 175, 621-630
   Abstract »    Full Text »    PDF »
beta-Sheet core of human prion protein amyloid fibrils as determined by hydrogen/deuterium exchange.
X. Lu, P. L. Wintrode, and W. K. Surewicz (2007)
PNAS 104, 1510-1515
   Abstract »    Full Text »    PDF »
Prion Interference Is Due to a Reduction in Strain-Specific PrPSc Levels.
J. C. Bartz, M. L. Kramer, M. H. Sheehan, J. A. L. Hutter, J. I. Ayers, R. A. Bessen, and A. E. Kincaid (2007)
J. Virol. 81, 689-697
   Abstract »    Full Text »    PDF »
Test for Detection of Disease-Associated Prion Aggregate in the Blood of Infected but Asymptomatic Animals.
B. Chang, X. Cheng, S. Yin, T. Pan, H. Zhang, P. Wong, S.-C. Kang, F. Xiao, H. Yan, C. Li, et al. (2007)
Clin. Vaccine Immunol. 14, 36-43
   Abstract »    Full Text »    PDF »
Prion infectivity in variant Creutzfeldt-Jakob disease rectum.
J D F Wadsworth, S Joiner, K Fox, J M Linehan, M Desbruslais, S Brandner, E A Asante, and J Collinge (2007)
Gut 56, 90-94
   Abstract »    Full Text »    PDF »
Strain Fidelity of Chronic Wasting Disease upon Murine Adaptation.
C. J. Sigurdson, G. Manco, P. Schwarz, P. Liberski, E. A. Hoover, S. Hornemann, M. Polymenidou, M. W. Miller, M. Glatzel, and A. Aguzzi (2006)
J. Virol. 80, 12303-12311
   Abstract »    Full Text »    PDF »
Transmission and characterization of bovine spongiform encephalopathy sources in two ovine transgenic mouse lines (TgOvPrP4 and TgOvPrP59).
C. Cordier, A. Bencsik, S. Philippe, D. Betemps, F. Ronzon, D. Calavas, C. Crozet, and T. Baron (2006)
J. Gen. Virol. 87, 3763-3771
   Abstract »    Full Text »    PDF »
Accelerated Accumulation of Misfolded Prion Protein and Spongiform Degeneration in a Drosophila Model of Gerstmann-Straussler-Scheinker Syndrome.
B. A. Gavin, M. J. Dolph, N. R. Deleault, J. C. Geoghegan, V. Khurana, M. B. Feany, P. J. Dolph, and S. Supattapone (2006)
J. Neurosci. 26, 12408-12414
   Abstract »    Full Text »    PDF »
Ultra-efficient Replication of Infectious Prions by Automated Protein Misfolding Cyclic Amplification.
P. Saa, J. Castilla, and C. Soto (2006)
J. Biol. Chem. 281, 35245-35252
   Abstract »    Full Text »    PDF »
Junctional expression of the prion protein PrPC by brain endothelial cells: a role in trans-endothelial migration of human monocytes.
P. Viegas, N. Chaverot, H. Enslen, N. Perriere, P.-O. Couraud, and S. Cazaubon (2006)
J. Cell Sci. 119, 4634-4643
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Early and Rapid Engraftment of Bone Marrow-Derived Microglia in Scrapie..
J. Priller, M. Prinz, M. Heikenwalder, N. Zeller, P. Schwarz, F. L. Heppner, and A. Aguzzi (2006)
J. Neurosci. 26, 11753-11762
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PrP genotypes of atypical scrapie cases in Great Britain..
G. C. Saunders, S. Cawthraw, S. J. Mountjoy, J. Hope, and O. Windl (2006)
J. Gen. Virol. 87, 3141-3149
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Cell-free formation of misfolded prion protein with authentic prion infectivity.
P. Weber, A. Giese, N. Piening, G. Mitteregger, A. Thomzig, M. Beekes, and H. A. Kretzschmar (2006)
PNAS 103, 15818-15823
   Abstract »    Full Text »    PDF »
Prion diseases in humans: an update.
R. BUTLER (2006)
The British Journal of Psychiatry 189, 295-296
   Abstract »    Full Text »    PDF »
A systematic review of prion therapeutics in experimental models.
C. R Trevitt and J. Collinge (2006)
Brain 129, 2241-2265
   Abstract »    Full Text »    PDF »
Codon 129 polymorphism of the human prion protein influences the kinetics of amyloid formation..
P. A. Lewis, M. H. Tattum, S. Jones, D. Bhelt, M. Batchelor, A. R. Clarke, J. Collinge, and G. S. Jackson (2006)
J. Gen. Virol. 87, 2443-2449
   Abstract »    Full Text »    PDF »
Cross-Talk between RNA and Prions.
C. G. Crist and Y. Nakamura (2006)
J. Biochem. 140, 167-173
   Abstract »    Full Text »    PDF »
Prion-induced amyloid heart disease with high blood infectivity in transgenic mice..
M. J. Trifilo, T. Yajima, Y. Gu, N. Dalton, K. L. Peterson, R. E. Race, K. Meade-White, J. L. Portis, E. Masliah, K. U. Knowlton, et al. (2006)
Science 313, 94-97
   Abstract »    Full Text »    PDF »
Childhood Onset in Familial Prion Disease With a Novel Mutation in the PRNP Gene..
E. Rogaeva, C. Zadikoff, J. Ponesse, G. Schmitt-Ulms, T. Kawarai, C. Sato, S. Salehi-Rad, P. St. George-Hyslop, and A. E. Lang (2006)
Arch Neurol 63, 1016-1021
   Abstract »    Full Text »    PDF »
Acid inactivation of prions: efficient at elevated temperature or high acid concentration..
T. R. Appel, R. Lucassen, M. H. Groschup, M. Joncic, M. Beekes, and D. Riesner (2006)
J. Gen. Virol. 87, 1385-1394
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Prion Proteins with Insertion Mutations Have Altered N-terminal Conformation and Increased Ligand Binding Activity and Are More Susceptible to Oxidative Attack.
S. Yin, S. Yu, C. Li, P. Wong, B. Chang, F. Xiao, S.-C. Kang, H. Yan, G. Xiao, J. Grassi, et al. (2006)
J. Biol. Chem. 281, 10698-10705
   Abstract »    Full Text »    PDF »
Conversion Efficiency of Bank Vole Prion Protein in Vitro Is Determined by Residues 155 and 170, but Does Not Correlate with the High Susceptibility of Bank Voles to Sheep Scrapie in Vivo.
N. Piening, R. Nonno, M. Di Bari, S. Walter, O. Windl, U. Agrimi, H. A. Kretzschmar, and U. Bertsch (2006)
J. Biol. Chem. 281, 9373-9384
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Functional disruption of the prion protein gene in cloned goats..
G. Yu, J. Chen, H. Yu, S. Liu, J. Chen, X. Xu, H. Sha, X. Zhang, G. Wu, S. Xu, et al. (2006)
J. Gen. Virol. 87, 1019-1027
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Characterization and Application of a Novel RNA Aptamer against the Mouse Prion Protein..
S. Sekiya, K. Noda, F. Nishikawa, T. Yokoyama, P. K.R. Kumar, and S. Nishikawa (2006)
J. Biochem. 139, 383-390
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Atypical prion protein in sheep brain collected during the British scrapie-surveillance programme.
S. J. Everest, L. Thorne, D. A. Barnicle, J. C. Edwards, H. Elliott, R. Jackman, and J. Hope (2006)
J. Gen. Virol. 87, 471-477
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Inactivation of Prions by Acidic Sodium Dodecyl Sulfate.
D. Peretz, S. Supattapone, K. Giles, J. Vergara, Y. Freyman, P. Lessard, J. G. Safar, D. V. Glidden, C. McCulloch, H.-O. B. Nguyen, et al. (2006)
J. Virol. 80, 322-331
   Abstract »    Full Text »    PDF »
Potential Role of Soil in the Transmission of Prion Disease.
P. T. Schramm, C. J. Johnson, N. E. Mathews, D. McKenzie, J. M. Aiken, and J. A. Pedersen (2006)
Reviews in Mineralogy and Geochemistry 64, 135-152
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Disease-related Prion Protein Forms Aggresomes in Neuronal Cells Leading to Caspase Activation and Apoptosis.
M. Kristiansen, M. J. Messenger, P.-C. Klohn, S. Brandner, J. D. F. Wadsworth, J. Collinge, and S. J. Tabrizi (2005)
J. Biol. Chem. 280, 38851-38861
   Abstract »    Full Text »    PDF »



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