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Life Sciences - Biochemistry & Biophysics | ADAMTS-13 - Biology and Disease


Biology and Disease

Rodgers, George (Ed.)

2015, X, 440 p. 8 illus., 4 illus. in color.

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ISBN 978-3-319-08717-7

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  • An up-to-date comprehensive overview of ADAMTS-13 basic science, including less-recognized aspects of the protein
  • Covers ADAMTS-13's role in disease, including its potential as a therapeutic agent
  • One of the only books to focus on this specific protein, it fills a gap in the literature and its coverage of both scientific and clinical aspects make it relevant both to researchers and clinicians

This comprehensive volume discusses the protease A13, summarizing the current status of basic and clinical research. Comprised of eight chapters, ADAMTS-13: Biology and Disease begins with an exploration of the biochemistry and structure-function relationships of A13 as well as its normal function in hemostasis (cleavage of von Willebrand factor). Emerging research themes for A13 are covered, including its potential role in angiogenesis and other aspects of cell biology. Clinical topics include laboratory assays for A13, inherited A13 deficiency, and acquired A13 deficiency. A chapter on related thrombotic microangiopathic (TMA) disorders examines the differences between TMAs associated with A13 deficiency and those not associated with A13 deficiency. Latter clinical chapters summarize treatment aspects, both standard and novel. A final chapter reviews the preliminary information on emerging aspects of A13, such as the status of recombinant A13 products and their potential utility. This is a significant resource for clinical hematologists, transfusion medicine physicians, and researchers interested in hemostasis, vascular biology, biochemistry, and metalloproteases.

Content Level » Research

Keywords » Angiogenesis - Protease - Thrombosis - Upshaw-Schulman - Von Willebrand

Related subjects » Biochemistry & Biophysics - Cell Biology - Internal Medicine

Table of contents 

1. A13: Structure and function.- 2. A13: The von Willebrand factor cleaving protease.- 3. A13: Angiogenesis and other biologic activities.- 4. A13:  Assays.- 5. Inherited A13 deficiency (Upshaw-Schulman Syndrome).- 6. Acquired A13 deficiency (TTP).- 7. Related thrombotic microangiopathies.- 8. Future directions.​

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