ADAMTS13 Biology and Disease
This comprehensive volume discusses the protease ADAMTS13, summarizing the current status of basic and clinical research. The nine authoritative chapters begin with a historical perspective followed by exploration of the biochemistry and structure-functio
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ADAMTS13 Biology and Disease
ADAMTS13
George M. Rodgers Editor
ADAMTS13 Biology and Disease
Editor George M. Rodgers Division of Hematology University of Utah Health Sciences Center Salt Lake City, UT, USA
ISBN 978-3-319-08716-0 ISBN 978-3-319-08717-7 DOI 10.1007/978-3-319-08717-7
(eBook)
Library of Congress Control Number: 2015945104 Springer Cham Heidelberg New York Dordrecht London © Springer International Publishing Switzerland 2015 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. 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. The publisher, the authors and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, express or implied, with respect to the material contained herein or for any errors or omissions that may have been made. Printed on acid-free paper Springer International Publishing AG Switzerland is part of Springer Science+Business Media (www.springer.com)
Preface
Ninety-one years ago, in 1924, Dr. Eli Moschowitz, a pathologist at Beth Israel Hospital in New York City, reported the first recognized case of thrombotic thrombocytopenic purpura (TTP). The patient developed an acute illness and on laboratory evaluation had anemia and proteinuria, but the blood urea nitrogen and creatinine tests were normal. Over a period of several days, the patient developed progressive neurologic impairment and died. At autopsy, Dr. Moschowitz noted widespread hyaline thrombosis of the terminal arterioles and capillaries. In his publication, Dr. Moschowitz concluded that death “resulted from some powerful poison which had both agglutinative and hemolytic properties.” Almost 60 years elapsed before Moake and colleagues described unusually large von Willebrand factor multimers in patients with TTP, and over a decade later, Furlan et al. reported deficiency of a von Willebrand factor “depolymerase” in TTP. Finally, in 2001 the vWF-cleaving protease was identified as ADAMTS13 (9); patients with inherited TTP have mutations in the ADAMTS13 gene, while patients with acquired TTP have antibodies to ADAMTS13. Thus, the “powerful poison” initially suggested by Dr. Moschowitz turns out to be ULvWF multimers, which in the absence of ADAMTS13 processing result in disseminated platelet thrombosis, thrombocytopenia, hemolysis, and organ d
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