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Prions and Brain Diseases in Animals and Humans

  • Book
  • © 1998

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Editors:
  1. Douglas R. O. Morrison

    1. Coppet, Switzerland

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Part of the book series: NATO Science Series A: (NSSA, volume 295)

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Table of contents (41 chapters)

  1. Front Matter

    Pages i-xiv
    Download chapter PDF

  2. Introduction — Guide to the Talks

    • Douglas R. O. Morrison
    Pages 1-18

  3. Polymorphic Genotype Matching in Acquired Creutzfeldt-Jakob Disease: An Analysis of Donor/Recipient Case Pairs

    • Paul Brown, Larisa Cervenáková, Lisa McShane, Paul Kleihues, Jean-François Foncin, George Collins et al.
    Pages 19-24

  4. Human Prion Protein Gene Mutation at Codon 183 Associated with an Atypical Form of Prion Disease

    • Ricardo Nitrini, Sergio Rosemberg, Maria Rita Passos-Bueno, Luis S. Texeira da Silva, Paula Iughetti, Maria Papadopoulos et al.
    Pages 25-32

  5. Fatal Familial Insomnia: A Human Model of Prion Disease

    • Elio Lugaresi, Pasquale Montagna, Pietro Cortelli, Paolo Tinuper, Patrizia Avoni, Giuseppe Plazzi
    Pages 33-35

  6. Mechanisms of Phenotypic Heterogeneity in Human Prion Diseases

    • Pierluigi Gambetti, Shu G. Chen, Piero Parchi
    Pages 37-41

  7. Transgenic Mice with Neuron-Specific Expression of a Hamster Prion Protein Minigene Are Susceptible to Hamster Scrapie Agent

    • Bruce Chesebro, Suzette A. Priola, Richard E. Race
    Pages 43-47

  8. The Use of Transgenic Mice in the Investigation of Transmissible Spongiform Encephalopathies

    • Charles Weissmann, Marek Fischer, Alex Raeber, Hans Ruedi Büeler, Andreas Sailer, Doron Shmerling et al.
    Pages 49-57

  9. Large-Scale Sequencing of Human, Mouse, and Sheep Prion Protein Genes

    • Inyoul Lee, David Westaway, Arian Smit, Carol Cooper, Hong Yao, Stanley B. Prusiner et al.
    Pages 59-75

  10. Electron Microscopy in Prion Research: Tubulovesicular Structures Are Not Composed of Prion Protein (PrP) but They May Be Intimately Associated with PrP Amyloid Fibrils

    • P. P. Liberski, M. Jeffrey, C. Goodsir
    Pages 77-86

  11. Familial Prion Diseases Modeled in Cell Culture

    • David A. Harris, Sylvain Lehmann, Nathalie Daude
    Pages 87-98

  12. Yeast Approach to Protein “Prionization”: SUP35-[PSI] System

    • S. G. Inge-Vechtomov, E. A. Ilmov, L. N. Mironova, V. L. Tikchomirova, K. V. Volkov, S. P. Zadorsky
    Pages 99-109

  13. Prions of Yeast: Genetic Evidence that the Non-Mendelian Elements, [PSI] and [URE3] Are Altered Self-Replicating Forms of Sup35p and Ure2p, Respectively

    • Reed B. Wickner, Daniel C. Masison, Herman Edskes, Marie-Lise Maddelein
    Pages 111-121

  14. Early Clinical Detection of Brain Diseases in Animals

    • B. F. Semenov, S. V. Ozherelkov, V. V. Vargin
    Pages 123-133

  15. Prion Biology and Diseases — Fatal Conformations of Proteins during a Journey from Heresy to Orthodoxy

    • Stanley B. Prusiner
    Pages 135-139

  16. New Variant Creutzfeldt-Jakob Disease

    • Robert Will
    Pages 141-145

  17. The Molecular Basis of Cellular Dysfunction in Prion Diseases

    • Randal R. Nixon, Yin Qiu, William Hyun, Stanley B. Prusiner, William C. Mobley, Stephen J. DeArmond
    Pages 147-158

  18. Presenilin Proteins and the Pathogenesis of Early-Onset Familial Alzheimer’s Disease: β-Amyloid Production and Parallels to Prion Diseases

    • D. Westaway, G. A. Carlson, C. Bergeron, G. Levesque, R. Sherrington, H. Yao et al.
    Pages 159-176

  19. Polyene Antibiotics in Experimental Transmissible Subacute Spongiform Encephalopathies

    • Vincent Beringue, Rémi Demaimay, Karim T. Adjou, Séverine Demart, François Lamoury, Michel Seman et al.
    Pages 177-185

  20. Scrapie Pathogenesis in Brain Grafts

    • Adriano Aguzzi, Sebastian Brandner
    Pages 187-195
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Keywords

About this book

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, indicating that Bovine Spongiform Encephalopathy, BSE or Mad Cow Disease, had penetrated the species barrier and was beginning to cause a new dis­ ease in humans--the new variant ofCreutzfeldt-Jakob Disease, nvCJD. This important and urgent subject became an additional major topic at the workshop. This is a book containing most of the talks plus the abstracts of those unable to find time to write up their talks. Almost all papers were written in the spring and summer of 1997 and contain material added after the workshop; thus Bob Will's paper on the new variant of cm contains data up to July 1997 and four contributions arrived in October 1997. In addition to the talks given at our workshop, there was a special joint session with the Planetary Emergencies Workshop where many distinguished scientists, including three Nobel laureates, discussed major issues affecting our planet. Six talks were given by us to this other workshop, five about prions, BSE, and nvCJD, and one on the broader issue of new epi­ demics by Luc Montagnier. Although most of the talks concerned research issues, there were a few special talks.

Editors and Affiliations

  • Coppet, Switzerland

    Douglas R. O. Morrison

Bibliographic Information

  • Book Title: Prions and Brain Diseases in Animals and Humans

  • Editors: Douglas R. O. Morrison

  • Series Title: NATO Science Series A:

  • DOI: https://doi.org/10.1007/978-1-4899-1896-3

  • Publisher: Springer New York, NY

  • eBook Packages: Springer Book Archive

  • Copyright Information: Springer Science+Business Media New York 1998

  • Hardcover ISBN: 978-0-306-45825-5Published: 31 March 1998

  • Softcover ISBN: 978-1-4899-1898-7Published: 06 June 2013

  • eBook ISBN: 978-1-4899-1896-3Published: 29 June 2013

  • Edition Number: 1

  • Number of Pages: XIV, 357

  • Topics: Biochemistry, general, Neurosciences

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