Mouse Models with Peroxisome Biogenesis Defects
Peroxisomes are ubiquitous organelles in mammalian cells but it is still unclear how they contribute to normal development and tissue homeostasis. To address this question, gene targeting techniques have been applied on several peroxins to interfere with
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lar machines Involved Machines involved in Peroxisome peroxisome biogenesis and Biogenesis maintenance Maintenance
Molecular Machines Involved in Peroxisome Biogenesis and Maintenance
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Ce´cile Brocard • Andreas Hartig Editors
Molecular Machines Involved in Peroxisome Biogenesis and Maintenance
Editors Ce´cile Brocard Mauerbach Austria
Andreas Hartig Max F. Perutz Laboratories University of Vienna Vienna Austria
ISBN 978-3-7091-1787-3 ISBN 978-3-7091-1788-0 (eBook) DOI 10.1007/978-3-7091-1788-0 Springer Wien Heidelberg New York Dordrecht London Library of Congress Control Number: 2014944309 # Springer-Verlag Wien 2014 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)
Preface
Peroxisomes are essential for life and mutations in genes coding for biogenesis factors are often associated with lethal disorders. Proteins required for the biogenesis of this organelle typically assemble in large molecular complexes, which participate in membrane formation, protein transport, peroxisome duplication, and inheritance during cell division. With their species-specific pathways such as the synthesis of ether lipid in mammals or the glyoxylate cycle in plants and yeasts, peroxisomes are multitasking organelles. The establishment of experimental strate
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