Lysosomes
Lysosomes are membrane-surrounded organelles which are present in all animal cells. The importance of this organelle is underlined by an increasing number of human diseases, which are associated with an impaired function of the lysosomal compartment. This
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Lysosomes Paul Saftig, Ph.D. Biochemisches Institut Christian-Albrechts-Universitat Kiel Kiel, Germany
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LYSOSOMES Medical Intelligence Unit Landes Bioscience / Eurekah.com Springer Science+Business Media, Inc. ISBN: 0-387-25562-1
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Copyright ©2005 Eurekah.com and Springer Science+Business Media, Inc. All rights reserved. This work may not be translated or copied in whole or in part without the written permission of the publisher, except for brief excerpts in connection with reviews or scholarly analysis. Use in connection with any form of information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed is forbidden. T h e use in the publication of trade names, trademarks, service marks and similar terms even if they are not identified as such, is not to be taken as an expression of opinion as to whether or not they are subject to proprietary rights. While the authors, editors and publisher believe that drug selection and dosage and the specifications and usage of equipment and devices, as set forth in this book, are in accord with current recommendations and practice at the time of publication, they make no warranty, expressed or implied, with respect to material described in this book. In view of the ongoing research, equipment development, changes in governmental regulations and the rapid accumulation of information relating to the biomedical sciences, the reader is urged to carefully review and evaluate the information provided herein. Springer Science+Business Media, Inc., 233 Spring Street, New York, New York 10013, U.S.A. http://www.springeronline.com Please address all inquiries to the Publishers: Landes Bioscience / Eurekah.com, 810 South Church Street, Georgetown, Texas 78626, U.S.A. Phone: 512/ 863 7762; FAX: 512/ 863 0081 http://www.eurekah.com http://www.landesbioscience.com Printed in the United States of America. 9 8 7 6 5 4 3 2 1
Library of Congress Cataloging-in-Publication Data Saftig, Paul. Lysosomes / Paul Saftig. p. ; cm. ~ (Medical intelligence unit) Includes bibliographical references and index. ISBN 0-387-25562-1 (alk. paper) 1. Lysosomes. I. Title. II. Series: Medical inteUigence unit (Unnumbered : 2003) [DNLM: 1. Lysosomes—enzymology. 2. Lysosomes—metabolism. 3. Biological Transport. 4. Lysosomal Storage Diseases—therapy. Q U 350 S128L 2005] QH603.L9S24 2005 571.6'55~dc22
2005013404
Dedicated to Jane and Lynn ... and all patients suffering of diseases with an impaired lysosomal function.
CONTENTS Preface 1. History and Morphology of the Lysosome Renate Lullmann-Rauch Morphology of Lysosomes History of the Lysosome Concept 2. Transport of Lysosomal Enzymes Stephan Storch and Thomas Braulke Synthesis and Modifications of Soluble Lysosomal Proteins Formation of Mannose 6-Phosphate Recognition Marker Mannose 6-Phosphate Receptors Signal Structur
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