Prions Prions Prions
A series of remarkable discoveries in the past three decades have led to the molecular and genetic characterization of the transmissible pathogen causing scrapie in animals and a quartet of human illnesses: kuru, Jakob-Creutzfeld disease, Gerstmann-Sträus
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Editors A. Capron, Lille . R.W. Com pans, Atlanta/Georgia M. Cooper, Birmingham/Alabama· H. Koprowski, Philadelphia/Pennsylvania· I. McConnell, Edinburgh F. Melchers, Basel· M. Oldstone, La Jolla/California S. Olsnes, Oslo· M. Potter, Bethesda/Maryland H. Saedler, Cologne· P.K. Vogt, La Jolla/California H. Wagner, Munich· I. Wilson, La Jolla/California
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Prions Prions Prions Edited by S.B. Prusiner
With 42 Figures
,
Springer
STANLEY B. PRUSINER, M.D.
Department of Neurology HSE 781 University of California San Francisco, CA 94 143-0518
USA
Cover illustration: Molecular models of the cellular prion protein (left panel) and the scrapie protein (right panel). (Prepared by Zewei Huang and Fred Cohen.) Cover design: K{)nkel+Lopka, /lvesheim
ISSN 0070-217X ISB N-13 : 978-3-642-64640-9 e-ISBN -13: 978-3-642-60983-1 DOl : 10.1007 /978-3-642-60983-1 This work is subject to copyright. All rights are reserved. whe ther the whole or part of the material is concerned, specifically the rights of translation, re printing, reuse of illustrations, recitation, broadcasting, reproduction on microfilm or in any other way, and storage in data banks. Duplication of this publication or parts thereof is permitted only under the provisions of the German Copyrigh t Law of September 9, 1965, in its current version, and pe rmission for use must always be obtained from Springer-Verlag. Violations are liable for prosecution under the German Copyright law. Cl Springer-Verlag Berlin Heidelberg 1996 Softcover reprint of the hardcover 1st edition 1996 library of Congress Catalog Card Number 15-1 2910 The use of general descriptive names, registered names, trademarks, etc. in this publication does not imply, even in the absence of a spec ific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. Product liability: The publishers cannot guaran tee the accuracy of any information about dosage and application contained in this book. In every individual case the user must check such informa tion by consulting other re levant literature. Typesetting: Thomson Press (Ind ia) ltd. Madras SPIN: 10541812 27/3020/SPS - 5 4 3 2 1 Printed on acid-free paper
list of Contents
S.B. PRUSINER Human Prion Diseases and Neurodegeneration ...... . P. GAMBETII Fatal Familial Insomnia and Familial Creutzfeldt-Jakob Disease: A Tale of Two Diseases with the Same Genetic Mutation
19
T. KITAMOTO and J. TATEISHI Human Prion Disease and Human Prion Protein Disease
27
G.A. CARLSON Prion Strains . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
35
Z. HUANG, S.B. PRUSINER, and F.E. COHEN Structures of Prion Proteins and Conformational Models for Prion Diseases . . . . . . .
49
J. SAFAR The Folding Intermediate Concept of Prion Protein Formation and Conformational Links to Infectivity. . . . . . . . . . . . . .
69
D.A. HARRIS, A. GORODINSKY, S. LEHMANN, K. MOULDE
