| Introduction -- Guide to the Talks |
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1 | (18) |
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Polymorphic Genotype Matching in Acquired Creutzfeldt-Jakob Disease: An Analysis of Donor/Recipient Case Pairs |
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19 | (6) |
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Human Prion Protein Gene Mutation at Codon 183 Associated with an Atypical Form of Prion Disease |
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25 | (8) |
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Fatal Familial Insomnia: A Human Model of Prion Disease |
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33 | (4) |
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Mechanisms of Phenotypic Heterogeneity in Human Prion Diseases |
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37 | (6) |
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Transgenic Mice with Neuron-Specific Expression of a Hamster Prion Protein Minigene Are Susceptible to Hamster Scrapie Agent |
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43 | (6) |
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The Use of Transgenic Mice in the Investigation of Transmissible Spongiform Encephalopathies |
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49 | (10) |
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Large-Scale Sequencing of Human, Mouse, and Sheep Prion Protein Genes |
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59 | (18) |
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Electron Microscopy in Prion Research: Tubulovesicular Structures Are Not Composed of Prion Protein (PrP) but They May Be Intimately Associated with PrP Amyloid Fibrils |
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77 | (10) |
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Familial Prion Diseases Modeled in Cell Culture |
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87 | (12) |
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Yeast Approach to Protein "Prionization": SUP35-[PSI] System |
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99 | (12) |
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Prions of Yeast: Genetic Evidence that the Non-Mendelian Elements, [PSI] and [URE3] Are Altered Self-Replicating Forms of Sup35p and Ure2p, Respectively |
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111 | (12) |
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Early Clinical Detection of Brain Diseases in Animals |
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123 | (12) |
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Prion Biology and Diseases -- Fatal Conformations of Proteins during a Journey from Heresy to Orthodoxy |
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135 | (6) |
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New Variant Creutzfeldt-Jakob Disease |
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141 | (6) |
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The Molecular Basis of Cellular Dysfunction in Prion Diseases |
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147 | (12) |
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Presenilin Proteins and the Pathogenesis of Early-Onset Familial Alzheimer's Disease: Beta-Amyloid Production and Parallels to Prion Diseases |
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159 | (18) |
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Polyene Antibiotics in Experimental Transmissible Subacute Spongiform Encephalopathies |
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177 | (10) |
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Scrapie Pathogenesis in Brain Grafts |
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187 | (10) |
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Structural Properties of Recombinant Human Prion Protein |
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197 | (6) |
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Autonomous Folding and Three-Dimensional Structure of the Carboxy-Terminal Domain of the Mouse Prion Protein, PrP (121-231) |
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203 | (14) |
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Protease-Resistant Prion Protein Formation |
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217 | (8) |
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Biophysical Studies on Structure Structural Transitions and Infectivity of the Prion Protein |
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225 | (20) |
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Amyloidogenesis in Transmissible Spongiform Encephalopathies |
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245 | (8) |
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Neuronal Degeneration and Cell Death in Prion Disease |
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253 | (16) |
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Clinical, Pathological, and Molecular Characterization of Gerstmann-Straussler-Scheinker Disease in the Indiana Kindred (PRNP F198S) |
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269 | (10) |
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Studies on the Pathogenesis of Scrapie and the Purification of Scrapie Agent in the Hamster Model |
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279 | (6) |
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PrP Peptides as a Tool to Investigate the Pathogenesis of Prion Protein Amyloidoses |
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285 | (6) |
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Properties of the Prion Proteins in Creutzfeldt Jakob Disease Patients Heterozygous for the E200K Mutation |
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291 | (6) |
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Strain Variation in Scrapie and BSE |
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297 | (2) |
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Transmission Studies of Fatal Familial Insomnia |
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299 | (4) |
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Molecular, Genetic, and Transgenetic Studies of Human Prion Disease |
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303 | (2) |
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Molecular Biology of Prion Propagation |
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305 | (2) |
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Ultrastructural and Immunocytochemical Studies on Prion Pathogenesis |
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307 | (2) |
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Overview of the BSE Epidemic |
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309 | (2) |
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Assessing Risks of BSE Transmission to Humans |
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311 | (2) |
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Human Activities Are Causing Selection of Pathogenic Agents |
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313 | (2) |
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Pathogenic Similarity of Slow Infections, Induced by Prions and Virions |
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315 | (2) |
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Protein Floding and Misfolding |
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317 | (2) |
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CJD Risk Factors: Analysis of 104 Patients |
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319 | (2) |
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Concluding Talk: Prions from a Physicist's Viewpoint -- Is the `Protein Only' Hypothesis Correct? |
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321 | (20) |
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| List of Speakers |
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341 | (2) |
| Names and Addresses of Participants |
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343 | (10) |
| Index |
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353 | |