Prions Methods and Protocols
This volume details protocols on prion disease from multiple disciplines and highlights the contribution each discipline has made to the understanding of prion disease. Written in the highly successful Methods in Molecular Biology series format, chapters
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Victoria A. Lawson Editor
Prions Methods and Protocols
METHODS
IN
MOLECULAR BIOLOGY
Series Editor John M. Walker School of Life and Medical Sciences University of Hertfordshire Hatfield, Hertfordshire, AL10 9AB, UK
For further volumes: http://www.springer.com/series/7651
Prions Methods and Protocols
Edited by
Victoria A. Lawson Department of Pathology The University of Melbourne Parkville, VIC, Australia
Editor Victoria A. Lawson Department of Pathology The University of Melbourne Parkville, VIC, Australia
ISSN 1064-3745 ISSN 1940-6029 (electronic) Methods in Molecular Biology ISBN 978-1-4939-7242-5 ISBN 978-1-4939-7244-9 (eBook) DOI 10.1007/978-1-4939-7244-9 Library of Congress Control Number: 2017952947 © Springer Science+Business Media LLC 2017 This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. The publisher, the authors and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, express or implied, with respect to the material contained herein or for any errors or omissions that may have been made. The publisher remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. Printed on acid-free paper This Humana Press imprint is published by Springer Nature The registered company is Springer Science+Business Media LLC The registered company address is: 233 Spring Street, New York, NY 10013, U.S.A.
Preface The transmissible spongiform encephalopathy or prion diseases are a group of invariably fatal neurodegenerative disorders with sporadic, familial and acquired etiologies that affect a variety of animal species including humans. It is now generally accepted that prion diseases arise through the aberrant misfolding of the host-encoded cellular prion protein. Protein misfolding, although not unique amongst neurodegenerative disorders, can in the case of prion diseases be seeded by the misfolded protein, a property which imparts the transmissible nature of prion diseases in the absence of a conventional infectious agent. It is this feature, in conjunction with difficulties associated with decontamination of infected surgical instruments and material, which can be attributed to the level of community interest and public health
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