MOLECULAR BIOLOGY INTELUGENCE UNIT The Biogenesis of Cellular Organelles Chris Mullins, Ph.D. Division of Kidney, Urologic and Hematologic Diseases National Institute of Diabetes and Digestive and Kidney Diseases National Institutes of Health Bethesda, Maryland, U.S.A. LANDES BIOSCIENCE / EUREKAH.COM KLUWER ACADEMIC / PLENUM PUBLISHERS GEORGETOWN, TEXAS NEW YORK, NEW YORK U.S.A. U.SA THE BIOGENESIS OF CELLULAR ORGANELLES Molecular Biology Intelligence Unit Landes Bioscience / Eurekah.com Kluwer Academic / Plenum Publishers Copyright ©2005 Eurekah.com and Kluwer Academic / Plenum Publishers All rights reserved. No part of this book may be reproduced or transmitted in any form or by any means, electronic or mechanical, including photocopy, recording, or any information storage and retrieval system, without permission in writing from the publisher. Printed in the U.S.A. Kluwer Academic / Plenum Publishers, 233 Spring Street, New York, New York, U.S.A. 10013 http://www.wkap.nl/ Please address all inquiries to the Publishers: Eurekah.com / Landes Bioscience, 810 South Church Street, Georgetown, Texas, U.S.A. 78626 Phone: 512/ 863 7762; FAX: 512/ 863 0081 http://www.eurekah.com http: //www.landesbioscience. com ISBN: 0-306-47990-7 The Biogenesis of Cellular Organelles, edited by Chris MuUins, Landes / Kluwer dual imprint / Landes series: Molecular Biology Intelligence Unit 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 recommend­ ations 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 carefiilly review and evaluate the information provided herein. Library of Congress Cataloging-in-Publication Data The biogenesis of cellular organelles / [edited by] Chris MuUins. p. ; cm. — (Molecular biology intelligence unit) Includes index. ISBN 0-306-47990-7 1. Cell organelles—Formation. I. MuUins, Chris. II. Series: Molecular biology intelligence unit (Unnumbered) [DNLM: 1. Evolution, Molecular. 2. Organelles. QH 591 B615 2004] QH581.2.B55 2004 571.6'5-dc22 2004019014 CONTENTS Preface vii 1. Theory of Organelle Biogenesis: A Historical Perspective 1 Barbara M. Mullock and J. PaulLuzio Definitions 1 The History of Organelle Recognition 2 Protein Synthesis and Targeting 4 Organization into Complex Structures 10 Organelle Inheritance 11 Challenges 13 2. Protein Coats As Mediators of Intracellular Sorting and Organelle Biogenesis 19 Chris Mullins Clathrin: A Scaffold for Protein Coats 22 Adaptor Protein Complexes: Adaptors for Coats of the Late-Secretory and Endocytic Pathways 25 COP Complexes: Protein Coats of the Early Secretory Pathway 29 Adaptor-Related Proteins Define Novel Coats of the Secretory and Endocytic Pathways 32 3. The Role of Proteins and Lipids in Organelle Biogenesis in the Secretory Pathway 45 Thomas F. J. Martin Protein Sorting Confers a Transient Nature to Secretory Pathway Organelles AG The Molecular Machinery Regulating Compartment Identity AG General Mechanisms Employed for Cargo Exit and Entry: Fission and Fusion 50 Exit Mechanisms in Trafficking 52 Entry Mechanisms in Trafficking: Tethering, Priming and Fusion 55 4. Endoplasmic Reticulum Biogenesis: Proliferation and Differentiation 63 Erik Snapp ER Functions 65 Building Blocks of the ER 69 ER Biogenesis 73 ER Network Formation 7A ERSubdomains 80 ER Differentiation 81 Putting It All Together 84 Appendices 85 5. The Golgi Apparatus: Structure, Ftmction and Cellular Dynamics 96 Nihal Altan-Bonnet and Jennifer LippincoU-Schwartz Golgi Structure and Distribution 96 Golgi Function and Compartmentalization 99 Transport within the Golgi Complex 101 Golgi Dynamics: Interphase 102 Golgi Dynamics: Mitosis 104 Golgi As a Scaffold for Signaling Molecules 104 6. Lysosome Biogenesis and Dynamics Ill Diane McVey Ward, Shelly L. Shiflett and Jerry Kaplan Models for Lysosome Biogenesis 112 Synthesis and Delivery of Lysosomal Hydrolases 113 Synthesis and Trafficking of Lysosomal Membrane Proteins 114 Lysosomes and Endosomes Undergo Fusion and Fission 115 Lysosomes Are Capable of Fusion with the Plasma Membrane 117 Movement of Lysosomes 118 7. Nucleogenesis 127 Sui Htcang The Nuclear Envelope in Mitosis 128 Post-Mitotic Biogenesis of the Nucleolus 132 8. Mitochondrial Biogenesis 138 Danielle Leuenbergery Sean P. Curran and Carla M. Koehler Mitochondrial Dynamics 139 Mitochondrial Protein Import 142 Mitochondrial Protein Export 150 The Protein Surveillance System of the Mitochondrion 150 Metal Ion Transport 152 9. The Biogenesis and Cell Biology of Peroxisomes in Human Health and Disease 164 Stanley R. Terlecky and Paul A. Walton Biogenesis of Peroxisomal Membranes 166 Early Acting Peroxins 168 The "Preperoxisome": A Precursor Organelle to the Peroxisome? 169 Molecular Mechanisms of Peroxisomal Protein Import 170 Index 177 EDITOR Chris Mullins, Ph.D. Division of Kidney, Urologic, and Hematologic Diseases National Institute of Diabetes and Digestive and Kidney Diseases National Institutes of Health Bethesda, Maryland, U.S.A. Chapter 2 CONTRIBUTORS Nihal Altan-Bonnet, Ph.D. Danielle Leuenberger, Ph.D. Cell Biology and Metabolism Branch Department of Chemistry National Institute of Child Health and Biochemistry and Human Development University of California, Los Angeles National Institutes of Health Los Angeles, California, U.S.A. Bethesda, Maryland, U.S.A. Chapter 8 Chapters Jennifer Lippincott-Schwartz, Ph.D. Sean P. Curran, Ph.D. Cell Biology and Metabolism Branch Department of Chemistry National Institute of Child Health and Biochemistry and Human Development University of California, Los Angeles National Institutes of Health Los Angeles, California, U.S.A. Bethesda, Maryland, U.S.A. Chapter 8 Chapter 5 Sui Huang, Ph.D. J. Paul Luzio, Ph.D. Department of Cell and Molecular Department of Clinical Biochemistry Biology Cambridge Institute Northwestern University Medical School for Medical Research Chicago, Illinois, U.S.A. University of Cambridge Chapter 7 Addenbrooke's Hospital Cambridge, United Kingdom Jerry Kaplan, Ph.D. Chapter 1 Department of Pathology University of Utah School of Medicine Thomas F. J. Martin, Ph.D. Salt Lake City, Utah, U.S.A. Department of Biochemistry Chapter 6 University of Wisconsin Madison, Wisconsin, U.S.A. Carla M. Koehler, Ph.D. Chapter 3 Department of Chemistry and Biochemistry Diane McVey Ward, Ph.D. University of California, Los Angeles Department of Pathology Los Angeles, California, U.S.A. University of Utah School of Medicine Chapter 8 Salt Lake City, Utah, U.S.A. Chapter 6 Barbara M. Mullock, Ph.D. Stanley R. Terlecky, Ph.D. Department of Clinical Biochemistry Department of Pharmacology Cambridge Institute Wayne State University School for Medical Research of Medicine University of Cambridge Detroit, Michigan, U.S.A. Addenbrooke's Hospital Chapter 9 Cambridge, United Kingdom Chapter 1 Paul A. Walton, Ph.D. Department of Anatomy and Cell Shelly L.Shiflett Biology Department of Pathology University of Western Ontario University of Utah School of Medicine London, Ontario, Canada Salt Lake City, Utah, U.S.A. Chapter 9 Chapter 6 Erik Snapp, Ph.D. Cell Biology and Metabolism Branch National Institute of Child Health and Human Development National Institutes of Health Bethesda, Maryland, U.S.A. Chapter 4 PREFACE -- The evolution of modern cell biology tools, such as confocal imaging techniques and advanced electron microscopy methodologies, has allowed for ever improving structural and functional characterizations of the cell. Such methods complement classical genetics and biochemistry in the ongoing effort to define cellular science. This is especially apparent in the area of organelle biology. Studies dating back over 100 years to the present have revealed the elaborate collection of distinctive membrane-bound cytoplasmic subcompartments, termed organelles, within the eukaryotic cell and defined their roles in mediating numerous specialized functions in cellular physiology. Organelles play an essential role in the cell in large part through ensuring a tight regulatory and functional separation of distinct chemical reactions, such as cellular respiration, and molecular processes, such as protein degradation and DNA replication. Many organelles are common to virtually all cell types (e.g., the nucleus) while others reside only in certain differentiated cells (e.g., the lysosome-related lytic granules and melanosomes found in cytotoxic T lymphocytes and melanocytes, respectively). The unique characteristics of such heterogeneous cellular organelles are dictated by their particular bio­ chemical composition and complement of biomolecules. The Biogenesis of Cellular Organelles seeks to describe the cellular and molecular mechanisms mediating the biogenesis, maintenance, and func­ tion of key eukaryotic organelles. This work consists of an initial discussion of the evolution of organelle biogenesis theory from early studies through re­ cent findings, overviews of the prominent cellular machineries involved in the biogenesis and maintenance of cellular organelles, and reviews of the function and biogenesis of a number of key organelles common to nearly all eukaryotic cells, including the endoplasmic reticulum, the Golgi apparatus, the lysosome,