A Structural Perspective on Respiratory Complex I Structure and Func
Complex I (NADH:ubiquinone oxidoreductase) is the first and largest enzyme of the respiratory chain in mitochondria and plays a central role in cellular energy production. Complex I dysfunction has been implicated in many human neurodegenerative diseases,
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Leonid Sazanov Editor
A Structural Perspective on Respiratory Complex I Structure and Function of NADH:ubiquinone oxidoreductase
Editor Leonid Sazanov Medical Research Council Mitochondrial Biology Unit Wellcome Trust/MRC Building, Hills Road Cambridge CB2 0XY, UK
ISBN 978-94-007-4137-9 ISBN 978-94-007-4138-6 (eBook) DOI 10.1007/978-94-007-4138-6 Springer Dordrecht Heidelberg New York London Library of Congress Control Number: 2012938257 © Springer Science+Business Media Dordrecht 2012 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. Exempted from this legal reservation are brief excerpts in connection with reviews or scholarly analysis or material supplied specifically for the purpose of being entered and executed on a computer system, for exclusive use by the purchaser of the work. Duplication of this publication or parts thereof is permitted only under the provisions of the Copyright Law of the Publisher’s location, in its current version, and permission for use must always be obtained from Springer. Permissions for use may be obtained through RightsLink at the Copyright Clearance Center. Violations are liable to prosecution under the respective Copyright Law. 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. While the advice and information in this book are believed to be true and accurate at the date of publication, neither the authors nor the editors nor the publisher can accept any legal responsibility for any errors or omissions that may be made. The publisher makes no warranty, express or implied, with respect to the material contained herein. Printed on acid-free paper Springer is part of Springer Science+Business Media (www.springer.com)
Foreword
Complex I (NADH:ubiquinone oxidoreductase) is the first enzyme of the respiratory chain in mitochondria and bacteria. It is one of the largest and most elaborate membrane protein assemblies known. It plays a central role in cellular energy production, providing about 40% of the proton flux required for ATP synthesis. Complex I dysfunction has been implicated in many human neurodegenerative diseases and mutations in its subunits are the most common human genetic disorders known. Complex I is also a major source of reactive oxygen species in mitochondria, which may lead to Parkinson’s disease and could be involved in aging. The enzyme transfers two electrons from NADH to quinone, coupling this process to the translocation
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