K. Tanaka • E.W. Davie (Eds.) Y. Ikeda • S. Iwanaga • H. Saito • K. Sueishi (Associate Eds.) Recent Advances in Thrombosis and Hemostasis 2008 K. Tanaka • E.W. Davie (Eds.) Y. Ikeda • S. Iwanaga • H. Saito • K. Sueishi (Associate Eds.) Recent Advances in Thrombosis and Hemostasis 2008 Springer Editors Kenzo Tanaka, M.D., Ph.D., Professor Emeritus Department of Pathology, Kyushu University 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582, Japan Earl W. Davie, Ph.D., Professor Department of Biochemistry, University of Washington J317 Health Science Center, Box 357350, Seattle WA 98195, USA Associate editors Yasuo Ikeda, M.D., Ph.D., Professor Keio University, School of Medicine, Internal Medicine 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan Sadaaki Iwanaga, Ph.D., Professor Emeritus Department of Biology, Kyushu University 6-10-1 Hakozaki, Higashi-ku, Fukuoka 812-8581, Japan Hidehiko Saito, M.D., Ph.D., Professor Emeritus Department of Medicine, Nagoya University 26 Tsurumai-cho, Showa-ku, Nagoya 466-8550, Japan Katsuo Sueishi, M.D., Ph.D., Professor Pathophysiological and Experimental Division, Department of Pathology Kyushu University 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582, Japan Library of Congress Control Number: 2008923271 ISBN 978-4-431-78846-1 e-ISBN 978-4-431-78847-8 This work is subject to copyright. 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Springer is a part of Springer Science+Business Media springer.com © Springer 2008 Printed in Japan Typesetting: SNP Best-set Typesetter Ltd., Hong Kong Printing and binding: Kato Bunmeisha, Japan Printed on acid-free paper Preface Now more than ever, thrombotic and thromboembolic disorders as well as related diseases such as malignancies, arteriosclerosis, diabetes mellitus, hypertension, and obesity are the leading causes of morbidity and mortality. They have become urgent medical problems with serious economic consequences in industrialized and develop- ing countries alike. At the same time, the impact of molecular biology and genetics on our understanding of thrombosis and hemostasis is rapidly growing stronger as well as our knowledge of regeneration and development of specific tissues, organs, and embryos. Researchers are also constantly learning more about cardiovascular diseases as well as regulatory mechanisms for various intrinsic and extrinsic stimuli in viable tissues. In this volume, our intention has been to present the latest relevant information in molecular biology and genetics as well as the clinical implications of a better understanding of pathophysiology, novel diagnostic methodologies, and therapeutic applications for new methods of prevention in thrombosis/hemostasis and related disorders, including atherosclerosis. The dramatic advances in knowledge of thrombosis/hemostasis and vascular biology since the first publication of Recent Advances in Thrombosis and Fibrinolysis, edited with Japanese colleagues, in 1991, have required extensive revision in order to highlight and review recent progress in the field. The editors also gratefully welcome the seven distinguished non-Japanese authors, who, with their valuable contributions on subjects beyond the coverage by Japanese authors, have made this new edition truly international. We hope that this book will prove useful to readers who wish to expand their knowledge of the biology and medicine of thrombosis and hemostasis, thus justifying the effort and hard work of all those involved in its publication. We also note that the XXIIIrd International Congress of the International Society of Thrombosis and Haemostasis (ISTH) will be held in Kyoto, Japan, in July 2011. We earnestly hope that the endeavors that went into the publishing of this book will help to promote the scientific excitement that we anticipate for the 2011 ISTH Congress in Kyoto and will contribute to its greater success. March 2008 Kenzo Tanaka and Earl W. Davie, Editors Yasuo Ikeda, Sadaaki Iwanaga, Hidehiko Saito, and Katsuo Sueishi, Associate Editors Acknowledgments A special acknowledgment is due to the following colleagues who undertook the major task of coordinating various chapters and providing helpful critiques: Ikuro Maruyama, Osamu Matsuo, Yukio Ozaki, Yoichi Sakata, Koji Suzuki, and Akira Yoshioka (all from the Japanese Society of Thrombosis and Hemostasis). Our deepest appreciation also goes to the various contributors for their professional expertise and cooperation. Contents Preface V Contributors XI Part 1 Coagulation Mechanism Structure and Functions of Fibrinogen and Fibrin Michael W. Mosesson 3 Vitamin K Cycles and y-Carboxylation of Coagulation Factors Barrel W. Stafford and Christine M. Hebling 27 Synthesis and Secretion of Coagulation Factor VIII Michael U. Callaghan and Randal J. Kaufman 45 Structure and Biological Function of Factor XI Kazuo Fujikawa 68 Structural Insights into the Life History of Thrombin James A. Huntington 80 A Molecular Model of the Human Prothrombinase Complex Stephen J. Everse, Ty E. Adams, and Kenneth G. Mann 107 Lipid Mediators and Tissue Factor Expression Hiroyuki Takeya and Koji Suzuki 133 Tissue Factor Pathway Inhibitor: Structure and Function Hisao Kato 147 Biology of an Antithrombotic Factor—ADAMTS13 Fumiaki Banno and Toshiyuki Miyata 162 Antithrombin and Heparin Cofactor II: Structure and Functions Takehiko Koide 177 VII VIII Contents Part 2 Endothelial Cells and Inflammation High-Mobility Group Box i: Missing Link Between Thrombosis and Inflammation? Takashi Ito, Ko-ichi Kawahara, Teruto Hashiguchi, and Ikuro Maruyama ... 193 Coagulation, Inflammation, and Tissue Remodeling Koji Suzuki, Tatsuya Hayashi, Osamu Taguchi, and Esteban Gabazza 203 Structure and Function of the Endothelial Cell Protein C Receptor Kenji Fukudome 211 New Aspects of Antiinflamatory Activity of Antithrombin: Molecular Mechanism(s) and Therapeutic Implications Naoaki Harada and Kenji Okajima 218 Part 3 Platelets Platelet Collagen Receptors Stephanie M. Jung and Masaaki Moroi 231 Positive and Negative Regulation of Integrin Function Yoshiaki Tomiyama, Masamichi Shiraga, and Hirokazu Kashiwagi 243 GPIb-Related SignaHng Pathways in Platelets Yukio Ozaki 253 Sphingosine 1-Phosphate as a Platelet-Derived Bioactive Lipid Yutaka Yatomi 265 Polymorphisms of Platelet Membrane Glycoproteins Yumiko Matsubara, Mitsuru Murata, and Yasuo Ikeda 277 Platelet Procoagulant Activity Appeared by Exposure of Platelets to Blood Flow Conditions Shinya Goto 290 Part 4 Tissue Proteolysis Regulation of Cellular uPA Activity and Its Implication in Pathogenesis of Diseases Soichi Kojima 301 Role of tPA in the Neural System Nobuo Nagai and Tetsumei Urano 314 Role of Fibrinolysis in the Nasal System Takayuki Sejima and Yoichi Sakata 328 Role of Fibrinolysis in Hepatic Regeneration Kiyotaka Okada, Shigeru Ueshima, and Osamu Matsuo 336 Pathophysiology of Transglutaminases Including Factor FXIII Akitada Ichinose 348 Contents IX Part 5 Vascular Development and Regeneration A Linkage in the Developmental Pathway of Vascular and Hematopoietic Cells Jun K. Yamashita 363 Atherosclerosis and Angiogenesis: Double Face of Neovascularization in Atherosclerotic Intima and Collateral Vessels in Ischemic Organs Katsuo Sueishi, Mitsuho Onimaru, and Yutaka Nakashima 374 Part 6 Hemorrhagic Diathesis HemophiUa A and Inhibitors Midori Shima and Akira Yoshioka 389 von Willebrand Disease: Diagnosis and Treatment Masato Nishino 406 Part 7 Other Topics of Thrombosis and Hemostasis Age-Related Homeostasis and Hemostatic System Kotoku Kurachi, Sumiko Kurachi, Toshiyuki Hamada, Emi Suenaga, Tatiana Bolotova, and Elena Solovieva 427 Molecular Evolution of Blood Clotting Factors with Special Reference to Fibrinogen and von Willebrand Factor Sadaaki Iwanaga, Soutaro Gokudan, and Jun Mizuguchi 439 Snake Venoms and Other Toxic Components Affecting Thrombosis and Hemostasis Yasuo Yamazaki and Takashi Morita 462 Structures and Potential Roles of Oligosaccharides in Human Coagulation Factors and von Willebrand Factor Taei Matsui, Jiharu Hamako, and Koiti Titani 483 Part 8 Clinicals: Thrombosis-related Disorders and Recent Topics Hypercoagulable States Tetsuhito Kojima and Hidehiko Saito 507 Etiopathology of the Antiphospholipid Syndrome Tatsuya Atsumi, Olga Amengual, and Takao Koike 521 Malignancy and Thrombosis Hiroshi Kobayashi, Ryuji Kawaguchi, Yoriko Tsuji, Yoshihiko Yamada, Mariko Sakata, Seiji Kanayama, Shoji Haruta, and Hidekazu Oi 536 Disseminated Intravascular Coagulation Hideo Wada 551 Current Clinical Status of Venous Thromboembolism in Japan Mashio Nakamura, Takeshi Nakano, Satoshi Ota, Norikazu Yamada, Masatoshi Miyahara, Naoki Isaka, and Masaaki Ito 563 X Contents Platelet Activity and Antiplatelet Therapy in Patients with Ischemic Stroke and Transient Ischemic Attack Shinichiro Uchiyama, Tomomi Nakamura, Yumi Kimura, and Masako Yamazaki 574 Clinical Role of Recombinant Factor Vila in Bleeding Disorders Harold R. Roberts, Dougald M. Monroe, and Nigel S. Key 587 CHnical Role of Protein S Deficiency in Asian Population Naotaka Hamasaki and Taisuke Kanaji 597 Atherothrombosis and Thrombus Propagation Atsushi Yamashita and Yujiro Asada 614 Thrombotic Microangiopathy Yoshihiro Fujimura, Masanori Matsumoto, and Hideo Yagi 625 Keyword Index 641 Contributors Adams, Ty E. Department of Haematology, Division of Structural Medicine, Thrombosis Research Unit, Cambridge Institute for Medical Research, University of Cambridge Wellcome Trust/MRC, Cambridge CB2 2XY, UK Amengual, Olga Department of Medicine II, Hokkaido University Graduate School of Medicine N-15 W-7, Kita-ku, Sapporo 060-8638, Japan Asada, Yujiro Department of Pathology, Faculty of Medicine, University of Miyazaki 5200 Kihara, Kiyotake, Miyazaki-gun, Miyazaki 889-1692, Japan Atsumi, Tatsuya Department of Medicine II, Hokkaido University Graduate School of Medicine N-15 W-7, Kita-ku, Sapporo 060-8638, Japan Banno, Fumiaki Department of Vascular Physiology, National Cardiovascular Center Research Institute 5-7-1 Fujishiro-dai, Suita, Osaka 565-8565, Japan Bolotova, Tatiana Age Dimension Research Center, National Institute of Advanced Industrial Science and Technology (AIST) AIST Tsukuba Center 6-13, 1-1-1 Higashi, Tsukuba, Ibaraki 305-8566, Japan Callaghan, Michael U. Fellow Department of Pediatrics, Division of Hematology/Oncology, Children's Hospital of Michigan 3901 Beaubien Blvd., Detroit, MI 48201, USA XI
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