Proteostasis Methods and Protocols
This volume highlights the role of proteostasis in human health and associated disease model systems, reflecting its rising importance which has led to the development of new technologies to obtain insight into underling protein mechanistic events. Writte
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Rune Matthiesen Editor
Proteostasis 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
Proteostasis Methods and Protocols
Edited by
Rune Matthiesen Computational and Experimental Biology Group, Department of Health Promotion and Chronic Diseases, National Health Institute Dr. Ricardo Jorge, INSA, I.P., Lisboa, Portugal
Editor Rune Matthiesen Computational and Experimental Biology Group Department of Health Promotion and Chronic Diseases National Health Institute Dr. Ricardo Jorge, INSA, I.P. Lisboa, Portugal
ISSN 1064-3745 ISSN 1940-6029 (electronic) Methods in Molecular Biology ISBN 978-1-4939-3754-7 ISBN 978-1-4939-3756-1 (eBook) DOI 10.1007/978-1-4939-3756-1 Library of Congress Control Number: 2016939242 © Springer Science+Business Media New York 2016 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. Printed on acid-free paper This Humana Press imprint is published by Springer Nature The registered company is Springer Science+Business Media LLC New York
Preface Proteostasis or protein homeostasis is the process by which cells control the abundance and folding of the proteome. Proteostasis appears to be involved in many diseases and aging. For example, retinal dystrophies, neurodegenerative diseases, inflammatory diseases, infectious diseases, and cancer are broad categories of diseases already linked to proteostasis. For instance, several genes related to the ubiquitin pathway are implicated in retinal dystrophies. Retinal dystrophies are a group of rare diseases that affect individuals worldwide. Protein misfolding, aggregation, and accumulation are a common hallmark in various neurodegenerative diseases. The autophagy-lysosomal pathway and the ubiquitin-proteasome system, the two main intracellular degradation machineries, are essential for cell survival under stress cond
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