| Description |
1 online resource (306 p.) |
| Contents |
Cover -- Title Page -- Copyright Page -- Contents -- List of Contributors -- Foreword -- Preface -- Chapter 1 Historical perspective on von Willebrand disease -- Introduction -- The scientist of the disease -- First description of the disease: the Åland family -- Other early clinical reports -- The search for a new factor-the bleeding time factor -- The end of the beginning -- Recent scientific visits to Åland Islands -- References -- Chapter 2 Biosynthesis and organization of von Willebrand factor -- Introduction -- Terminology -- Molecular biology of VWF -- The VWF gene |
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VWF domain structure -- VWF promoter -- Cell biology of VWF -- VWF processing and dimerization in the endoplasmic reticulum -- VWF processing in the Golgi -- VWF multimerization -- Regulated storage of VWF in Weibel-Palade bodies and -granules -- Weibel-Palade body biogenesis -- Regulated release of VWF -- Postsecretion modification of VWF -- References -- Chapter 3 Von Willebrand factor structure and function -- Introduction -- VWF function -- Structure of VWF -- VWF domain structure -- Functional biochemistry of VWF -- Interaction of VWF with FVIII |
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Two sites of interaction between VWF and platelets -- Proteolysis of VWF -- Carbohydrate modifications of VWF -- Clearance of VWF through autoantibodies and other mechanisms -- Increased clearance as a cause of type 1C VWD -- Critical disulfide structure of VWF -- Shear and its effect on VWF -- Structure and function of the VWF propeptide -- Summary -- Acknowledgement -- References -- Chapter 4 Regulation of von Willebrand factor expression -- Introduction -- VWF gene structure and chromosomal location -- VWF expressing cells -- VWF promoter structure -- Transcriptional activators |
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Transcriptional suppressors -- NFY, a dual-purpose transcription factor -- Influence of regulatory sequence variants on VWF expression -- Regulatory variants in the proximal regulatory region: the VWF promoter -- Regulatory variants in the distal regulatory region: VWF enhancers -- Epigenetic regulation of VWF expression -- Posttranscriptional regulation of VWF expression -- Splicing events and differential VWF expression in endothelial cells -- MicroRNAs regulate VWF biosynthesis, maturation, and secretion -- Physiological, pathological, and environmental factors affecting VWF expression |
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Hemodynamic shear stress regulates VWF expression -- VWF is an acute-phase reactant -- Hormones raise blood VWF levels -- Conclusion -- References -- Chapter 5 Modulation of VWF by ADAMTS13 -- Introduction -- VWF structure, synthesis, and function -- ADAMTS13 structure -- ADAMTS family proteases -- Prodomain -- Metalloprotease (MP) domain -- Disintegrin-like (Dis) domain -- Thrombospondin type 1 repeats (TSP) -- Cysteine-rich (Cys-rich) domain -- Spacer domain -- CUB domains -- ADAMTS13 conformation/latency -- Global latency -- Local latency -- ADAMTS13 function |
| Notes |
Description based upon print version of record |
|
Physiology and pathophysiology of the VWF-ADAMTS13 axis |
| Genre/Form |
Electronic books
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| Form |
Electronic book
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| Author |
Berntorp, Erik E
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Lillicrap, David
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Montgomery, Robert R
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| ISBN |
9781119419495 |
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1119419492 |
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