Prions and Brain Diseases in Animals and Humans

Most of the world's experts on prions met for a workshop in Erice in August 1996. The aim of the workshop was to discuss the fundamentals of the science ofprions. It was for­ tunate that so many could be present given the pressure that they were under because of the data presented in March 1996...

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Bibliographic Details
Other Authors: Morrison, Douglas R.O. (Editor)
Format: eBook
Language:English
Published: New York, NY Springer US 1998, 1998
Edition:1st ed. 1998
Series:NATO Science Series A:, Life Sciences
Subjects:
Online Access:
Collection: Springer Book Archives -2004 - Collection details see MPG.ReNa
Table of Contents:
  • Prions of Yeast: Genetic Evidence that the Non-Mendelian Elements, [PSI] and [URE3] Are Altered Self-Replicating Forms of Sup35p and Ure2p, Respectively
  • Early Clinical Detection of Brain Diseases in Animals
  • Prion Biology and Diseases — Fatal Conformations of Proteins during a Journey from Heresy to Orthodoxy
  • New Variant Creutzfeldt-Jakob Disease
  • The Molecular Basis of Cellular Dysfunction in Prion Diseases
  • Presenilin Proteins and the Pathogenesis of Early-Onset Familial Alzheimer’s Disease: ?-Amyloid Production and Parallels to Prion Diseases
  • Polyene Antibiotics in Experimental Transmissible Subacute Spongiform Encephalopathies
  • Scrapie Pathogenesis in Brain Grafts
  • Structural Properties of Recombinant Human Prion Protein
  • Autonomous Folding and Three-Dimensional Structure of the Carboxy-Terminal Domain of the Mouse PrionProtein, PrP (121–231)
  • Protease-Resistant Prion Protein Formation
  • Assessing Risks of BSE Transmission to Humans
  • Human Activities Are Causing Selection of Pathogenic Agents
  • Pathogenic Similarity of Slow Infections, Induced by Prions and Virions
  • Protein Folding and Misfolding
  • CJD Risk Factors: Analysis of 104 Patients
  • Concluding Talk: Prions from a Physicist’s Viewpoint — Is the ‘Protein Only’ Hypothesis Correct?
  • List of Speakers
  • Names and Addresses of Participants
  • — Guide to the Talks
  • Polymorphic Genotype Matching in Acquired Creutzfeldt-Jakob Disease: An Analysis of Donor/Recipient Case Pairs
  • Human Prion Protein Gene Mutation at Codon 183 Associated with an Atypical Form of Prion Disease
  • Fatal Familial Insomnia: A Human Model of Prion Disease
  • Mechanisms of Phenotypic Heterogeneity in Human Prion Diseases
  • Transgenic Mice with Neuron-Specific Expression of a Hamster Prion Protein Minigene Are Susceptible to Hamster Scrapie Agent
  • The Use of Transgenic Mice in the Investigation of Transmissible Spongiform Encephalopathies
  • Large-Scale Sequencing of Human, Mouse, and Sheep Prion Protein Genes
  • Electron Microscopy in Prion Research: Tubulovesicular Structures Are Not Composed of Prion Protein (PrP) but They May Be Intimately Associated with PrP Amyloid Fibrils
  • Familial Prion Diseases Modeled in Cell Culture
  • Yeast Approach to Protein “Prionization”: SUP35-[PSI] System
  • Biophysical Studies on Structure Structural Transitions and Infectivity of the Prion Protein
  • Amyloidogenesis in Transmissible Spongiform Encephalopathies
  • Neuronal Degeneration and Cell Death in Prion Disease
  • Clinical, Pathological, and Molecular Characterization of Gerstmann-Sträussler-Scheinker Disease in the Indiana Kindred (PRNP F198S)
  • Studies on the Pathogenesis of Scrapie and the Purification of Scrapie Agent in the Hamster Model
  • PrP Peptides as a Tool to Investigate the Pathogenesis of Prion Protein Amyloidoses
  • Properties of the Prion Proteins in Creutzfeldt Jakob Disease Patients Heterozygous for the E200K Mutation
  • Strain Variation in Scrapie and BSE
  • Transmission Studies of Fatal Familial Insomnia
  • Molecular, Genetic, and Transgenetic Studies of Human Prion Disease
  • Molecular Biology of Prion Propagation
  • Ultrastructural and Immunocytochemical Studies on Prion Pathogenesis
  • Overview of the BSE Epidemic