Prions and Brain Diseases in Animals and Humans

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  • Format: Hardcover
  • Copyright: 1998-04-01
  • Publisher: Plenum Pub Corp

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Proceedings of a NATO Advanced Research Workshop on Prions and Brain Diseases in Animals and Humans, held August 19-23, 1996, in Erice, Italy. 46 contributors, 11 U.S. DNLM: Prion Diseases--congresses.

Table of Contents

Introduction -- Guide to the Talks 1(18)
Douglas R. O. Morrison
Polymorphic Genotype Matching in Acquired Creutzfeldt-Jakob Disease: An Analysis of Donor/Recipient Case Pairs
Paul Brown
Larisa Cervenakova
Lisa McShane
Paul Kleihues
Jean-Francois Foncin
George Collins
Frank Bastian
Lev G. Goldfarb
D. Carleton Gajdusek
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
P.M. Carrilho
Paulo Caramelli
Steffen Albrecht
Mayana Zatz
Andrea LeBlanc
Fatal Familial Insomnia: A Human Model of Prion Disease
Elio Lugaresi
Pasquale Montagna
Pietro Cortelli
Paolo Tinuper
Patrizia Avoni
Giuseppe Plazzi
Mechanisms of Phenotypic Heterogeneity in Human Prion Diseases
Pierluigi Gambetti
Shu G. Chen
Piero Parchi
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
The Use of Transgenic Mice in the Investigation of Transmissible Spongiform Encephalopathies
Charles Weissmann
Marek Fischer
Alex Raeber
Hans Ruedi Bueler
Andreas Sailer
Doron Shmerling
Thomas Rulicke
Sebastian Brandner
Adriano Aguzzi
Large-Scale Sequencing of Human, Mouse, and Sheep Prion Protein Genes
Inyoul Lee
David Westaway
Arian Smit
Carol Cooper
Hong Yao
Stanley B. Prusiner
Leroy Hood
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
Familial Prion Diseases Modeled in Cell Culture
David A. Harris
Sylvain Lehmann
Nathalie Daude
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
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
Early Clinical Detection of Brain Diseases in Animals
B.F. Semenov
S.V. Ozherelkov
V.V. Vargin
Prion Biology and Diseases -- Fatal Conformations of Proteins during a Journey from Heresy to Orthodoxy
Stanley B. Prusiner
New Variant Creutzfeldt-Jakob Disease
Robert Will
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
Presenilin Proteins and the Pathogenesis of Early-Onset Familial Alzheimer's Disease: Beta-Amyloid Production and Parallels to Prion Diseases
David Westaway
G.A. Carlson
C. Bergeron
G. Levesque
R. Sherrington
H. Yao
R. Strome
B. Perry
A. Davies
S. Gandy
C. Weaver
P. Davies
D. Shenk
J. Rommens
J. Roder
P.E. Fraser
P. St. George-Hyslop
Polyene Antibiotics in Experimental Transmissible Subacute Spongiform Encephalopathies
Vincent Beringue
Remi Demaimay
Karim T. Adjou
Severine Demart
Francois Lamoury
Michel Seman
Corinne I. Lasmezas
Jean-Philippe Deslys
Dominique Dormont
Scrapie Pathogenesis in Brain Grafts
Adriano Aguzzi
Sebastian Brandner
Structural Properties of Recombinant Human Prion Protein
Graham Jackson
Andrew Hill
Catherine Joseph
Anthony Clarke
John Collinge
Autonomous Folding and Three-Dimensional Structure of the Carboxy-Terminal Domain of the Mouse Prion Protein, PrP (121-231)
Rudi Glockshuber
Simone Hornemann
Roland Riek
Gerhard Wider
Martin Billeter
Kurt Wuthrich
Protease-Resistant Prion Protein Formation
Byron Caughey
Gregory J. Raymond
Biophysical Studies on Structure Structural Transitions and Infectivity of the Prion Protein
Detlev Riesner
Klaus Kellings
Karin Post
Martin Pitschke
Holger Wille
Hana Serban
Darlene Groth
Michael A. Baldwin
Stanley B. Prusiner
Amyloidogenesis in Transmissible Spongiform Encephalopathies
Franco Cardone
Maurizio Pocchiari
Neuronal Degeneration and Cell Death in Prion Disease
Hans A. Kretzschmar
Armin Giese
Jochen W. Herms
David R. Brown
Clinical, Pathological, and Molecular Characterization of Gerstmann-Straussler-Scheinker Disease in the Indiana Kindred (PRNP F198S)
B. Ghetti
F. Tagliavini
S.R. Dlouhy
P. Piccardo
K. Young
R.D. Yee
G.D. Hutchins
G. Giaccone
F. Prelli
B. Frangione
F. Unverzagt
O. Bugiani
M.R. Farlow
Studies on the Pathogenesis of Scrapie and the Purification of Scrapie Agent in the Hamster Model
Heino Diringer
Elizabeth Baldauf
Michael Beekes
Muhsin Ozel
PrP Peptides as a Tool to Investigate the Pathogenesis of Prion Protein Amyloidoses
Fabrizio Tagliavini
Giorgio Giaccone
Frances Prelli
Blas Frangione
Mario Salmona
Gianluigi Forloni
Bernardino Ghetti
Orso Bugiani
Properties of the Prion Proteins in Creutzfeldt Jakob Disease Patients Heterozygous for the E200K Mutation
Ruth Gabizon
Glenn Telling
Zeev Meiner
Michele Halimi
Irit Kahana
Stanley B. Prusiner
Strain Variation in Scrapie and BSE
Moira E. Bruce
Transmission Studies of Fatal Familial Insomnia
Katie Sidle
Bob Will
Peter Lantos
John Collinge
Molecular, Genetic, and Transgenetic Studies of Human Prion Disease
J. Collinge
J. Beck
T.A. Campbell
M. Desbuslais
I. Gowland
A. Hill
S. Mahal
J. Meads
K.C.L. Sidle
J.G.R. Jefferys
Molecular Biology of Prion Propagation
Stanley B. Prusiner
Kiyotoshi Kaneko
Martin Vey
Glenn Telling
Michael Scott
Ruth Gabizon
Albert Taraboulos
Stephen J. De Armond
Fred E. Cohen
Ultrastructural and Immunocytochemical Studies on Prion Pathogenesis
Lajos Laszlo
Overview of the BSE Epidemic
John Wilesmith
Assessing Risks of BSE Transmission to Humans
John Collinge
Human Activities Are Causing Selection of Pathogenic Agents
Luc Montagnier
Pathogenic Similarity of Slow Infections, Induced by Prions and Virions
V. A. Zouev
Protein Floding and Misfolding
A. R. Clarke
CJD Risk Factors: Analysis of 104 Patients
Eva Mitrova
Concluding Talk: Prions from a Physicist's Viewpoint -- Is the `Protein Only' Hypothesis Correct?
Douglas R. O. Morrison
List of Speakers 341(2)
Names and Addresses of Participants 343(10)
Index 353

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