THE COMPLEMENT SYSTEM: Novel Roles in Health and Disease THE COMPLEMENT SYSTEM: Novel Roles in Health and Disease Edited by Janos Szebeni Walter Reed Army Institute of Research Silver Spring, MD KLUWER ACADEMIC PUBLISHERS NEW YORK,BOSTON, DORDRECHT, LONDON, MOSCOW eBookISBN: 1-4020-8056-5 Print ISBN: 1-4020-8055-7 ©2004 Springer Science + Business Media, Inc. Print ©2004 Kluwer Academic Publishers Boston All rights reserved No part of this eBook maybe reproducedor transmitted inanyform or byanymeans,electronic, mechanical, recording, or otherwise, without written consent from the Publisher Created in the United States of America Visit Springer's eBookstore at: http://www.ebooks.kluweronline.com and the Springer Global Website Online at: http://www.springeronline.com This book may provide relief for those “hopelessly infected with the C bug” Tibor Borsos, Chapter 1 CONTENTS Dedication v Contents vii List of Contributors xi Foreword Otto Götze xvii Preface Janos Szebeni xix THE COMPLEMENT SYSTEM NOVELROLES IN HEALTH AND DISEASE PART1. A PIECE OF COMPLEMENT HISTORY 1 1. The Development of the “One-Hit” or “Single Site” Theory of Complement Mediated Immune Hemolysis A Personal Account T. Borsos 3 PART2.PATHWAYS,GENETICS AND GENEREGULATION OF COMPLEMENT 17 2. The Initiation Complexes of the Classical and Lectin Pathways P. Gál, G. Ambrus, and P. Závodszky 19 3. Complement Genetics Gy. Ábel and V. Agnello 45 4. Regulation of Complement Receptor Gene Expression: Regulators and Inhibitors M. Tolnay and G. C. Tsokos 61 viii Complement in Health and Disease PART3.NOVELREGULATORYROLES OF COMPLEMENT IN HEALTH AND DISEASE 75 5. Coordination of AdaptiveImmune Responses by C3 A. Erdei, E. Molnár, E. Csomor, Zs. Bajtay and J. Prechl 77 6. Terminal Complement Complex: Regulation of Formation and Pathophysiological Functions F. Tedesco, R. Bulla and F. Fischetti 97 7. The Many Faces of the Membrane Regulators of Complement C. L. Harris and B. P. Morgan 129 8. New Insights Into the Regulation of Complement Activation by Decay Accelerating Factor L.Kuttner-Kondo and E. M. Medof 167 9. The Role of Complement in Pregnancy and Fetal Loss G. Girardi and J. E. Salmon 187 10. Complement Deficiencies: a 2004 Update Gy. Ábel and V. Agnello 201 PART4.EXPLOITATION OF COMPLEMENTPROTEINS IN INFECTION AND CANCER 229 11. Microbial Evasion Mechanisms Against Human Complement R. Würzner and P. F. Zipfel 231 12. Complement-Mediated Antibody-Dependent Enhancement of Viral Infections Z. Prohászka, F.D. Tóth, D. Bánhegyi and G. Füst 249 13. Tumor Cell Resistance to Complement-Mediated Lysis M. Kirschfink and Z. Fishelson 265 Contents ix PART5.ROLE OF COMPLEMENT IN AUTOIMMUNE DISEASES,ALLERGY AND TRANSPLANTATION 305 14. Complement and Autoimmunity G. C. Tsokos and M. Tolnay 307 15. The Complex Roles of Anaphylatoxins in Allergic Asthma and Autoimmune Diseases H. Hawlisch, M. Wills-Karp, C.L. Karp, and J. Köhl 315 16. Role of Complement in Allergy L. Varga, H. Farkas and G. Füst 345 17. Complement Activation-Related Pseudoallergy: Mechanism of Anaphylactoid Reactions to Radiocontrast Media and Drug Carrier Liposomes and Micelles J. Szebeni 361 18. The Role of Complement in Transplantation M. Kirschfink and T.E. Mollnes 397 PART6.ROLE OF COMPLEMENT IN ACUTECATASTROPHIC ILLNESSES 419 19. Role of Complement in Myocardial Ischemia and Infarction M.C. Walsh, M.L. Hart, T.M. Bourcier, D. Bhole, M. Takahashi and G.L. Stahl 421 20. Role of Complement in Intestinal Ischemia/Reperfusion Induced Injury S. D. Fleming and G. Tsokos 437 21. Role of C5a and C5a Receptor in Sepsis F.S. Zetoune, P.A. Ward and M. S. Huber-Lang 451 22. Role of Complement in Multi-Organ Dysfunction Syndrome M. S. Huber-Lang, J. V. Sarma, F. S. Zetoune and P.A. Ward 465 x Complement in Health and Disease PART7.THECOMPLEMENTSYSTEM AS THERAPEUTICAL TARGET 481 23. Therapeutic Manipulation of the Complement System T. E. Mollnes 483 24. Activation and Inhibition of Complement by Immunoglobulins M. Basta 517 25. Engineering Control of Complement Inhibition at the Cell Surface R.A.G. Smith, D. Esser, S. H. Ridley and R. Bradford 531 INDEX 547 CONTRIBUTORS Basta,Milan,M.D., Ph.D. National Institute for Ábel, György, M.D. Ph.D. Neurological Disorders and Medical Director, Department of Stroke; National Institutes of LaboratoryMedicine,Lahey Health, Bethesda, Maryland, U.S. Clinic Medical Center, [email protected] Burlington, MA, and Department of Pathology, Harvard Medical Bhole, Deepak School, Boston, MA, Postdoctoral fellow [email protected] Center for Experimental Therapeutics and Reperfusion Ambrus, Géza, Ph.D. Injury, Department of Postdoctoral Fellow Anesthesiology, Perioperative Institute of Enzymology and Pain Medicine Biological Research Center Brigham and Women’s Hospital, Hungarian Academy of Sciences Harvard Medical School, Budapest,Hungary Boston, MA, [email protected] Borsos, Tibor, Sc.D. Angello, Vincent, M.D. Scientist Emeritus Director, Immunology Research, 4620 N Park Ave Apt. 608E Department of Laboratory Chevy Chase, MD 20815, Medicine, Lahey Clinic Medical [email protected] Center, Burlington, MA, and Edith Nourse Rogers Bourcier, Todd M. VeteransAffairs Hospital Instructor, Center for Bedford, MA, Experimental Therapeutics and [email protected] Reperfusion Injury, Brigham and Women’s Hospital, Bajtay, Zsuzsa, Ph.D. Harvard Medical School, Res. Assoc..Prof. Boston, MA, Department of Immunology, Eötvös Loránd University, Bradford, Roberta, B.Sc., DIBT Budapest, Hungary Head of Pharmacology and Toxicology, Adprotech Ltd. Bánhegyi, Dénes, M.D. Cambridge, UK Head, Department of [email protected] Immunology, St László Hospital Budapest Bulla, Roberta, Ph.D. [email protected] Postdoctoral Fellow, Department of Physiology and Pathology University of Trieste, Italy xii Complement in Health and Disease Csomor, Eszter Fleming, Sherry, D. Department of Immunology, ResearchAss. Professor, Dept. of Eötvös Loránd University, Cellular Injury, Walter Reed Budapest, Hungary Army Inst. of Research and Dept. [email protected] of Medicine, Uniformed Services University of the HealthSciences, Erdei, Anna, Ph.D., D.Sc. Bethesda, MD, Professor, Head of Department [email protected]. and Research Group of the mil Hungarian Academy of Sciences, Eötvös Loránd Füst, George, M.D., Ph.D., D.Sc. University, Budapest,Hungary Professor, Chief, Research Anna.Erdei @ freemail.hu Laboratory, Third Department of Medicine, Faculty of Medicine Esser, Dirk, Ph.D. Semmelweis University, Budapest Senior Scientific Investigator Hungary AdprotechLtd., Cambridge, UK [email protected] [email protected] Gál, Péter, Ph.D. Farkas, Henriette, M.D., PhD Senior Scientist, Institute of Head, Angoedema and Allergy Enzymology, Biological Research Center, Semmelweis University, Center Hungarian Academy of Budapest, Hungary Sciences, Budapest, Hungary [email protected] [email protected] Fischetti, Fabio, M.D. Girardi, Guillermina, Ph.D. SeniorScientific Investigator Department of Medicine Department of Medicine and Hospital for Special Surgery Neurology, University of Trieste Weill Medical College of Cornell Trieste,Italy University, New York, N.Y. [email protected] Fishelson, Zvi, Ph.D. Professor, Department of Cell and Götze, Otto, M.D. Developmental Biology, Sackler Emeritus Professor of School of Medicine, Tel Aviv Immunology University, Tel Aviv, Israel Bodelschwingh Str. 20, [email protected] D 37075 Göttingen Ogoetze @ gwdg.de