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Chapter 113: Molecular Biology and Biochemistry of the Coagulation Factors and Pathways of Hemostasis

Mettine H. A. Bos; Cornelis van ‘t Veer; Pieter H. Reitsma

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    AMA Citation
    Bos MA, van ‘t Veer C, Reitsma PH. Bos M.A., van ‘t Veer C, Reitsma P.H. Bos, Mettine H. A., et al.Molecular Biology and Biochemistry of the Coagulation Factors and Pathways of Hemostasis. In: Kaushansky K, Lichtman MA, Prchal JT, Levi MM, Press OW, Burns LJ, Caligiuri M. Kaushansky K, Lichtman M.A., Prchal J.T., Levi M.M., Press O.W., Burns L.J., Caligiuri M Eds. Kenneth Kaushansky, et al.eds. Williams Hematology, 9e New York, NY: McGraw-Hill; . http://accessmedicine.mhmedical.com/content.aspx?bookid=1581&sectionid=108079516. Accessed January 18, 2019.
    MLA Citation
    Bos MA, van ‘t Veer C, Reitsma PH. Bos M.A., van ‘t Veer C, Reitsma P.H. Bos, Mettine H. A., et al.. "Molecular Biology and Biochemistry of the Coagulation Factors and Pathways of Hemostasis." Williams Hematology, 9e Kaushansky K, Lichtman MA, Prchal JT, Levi MM, Press OW, Burns LJ, Caligiuri M. Kaushansky K, Lichtman M.A., Prchal J.T., Levi M.M., Press O.W., Burns L.J., Caligiuri M Eds. Kenneth Kaushansky, et al. New York, NY: McGraw-Hill, , http://accessmedicine.mhmedical.com/content.aspx?bookid=1581&sectionid=108079516.

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INTRODUCTION

SUMMARY

The coagulation cascade consists of a complex network of reactions that are essential for the conversion of zymogens into enzymes and of inactive procofactors into cofactors. Most of these reactions take place on a membrane surface, which restricts coagulation to the site of injury. Upon initiation, these reactions serve to produce the fibrin that is necessary for the formation of a stable hemostatic plug. In addition, these reactions provide feedback loops that limit and localize thrombus formation and regulate thrombus resolution. This chapter highlights key biochemical characteristics of the individual coagulation factors, essential aspects regarding their synthesis, and the clinical importance of acquired or inherited variations that affect their quantity or function. The coagulation factors are grouped as (1) the vitamin K-dependent zymogens (prothrombin, factor VII, factor IX, factor X, and protein C); (2) the procoagulant cofactors (factor V, factor VIII); (3) the soluble cofactors (protein S, von Willebrand factor); (4) factor XI and the contact system (factor XII, prekallikrein, and high-molecular weight kininogen); (5) the cell-associated cofactors (tissue factor, thrombomodulin, endothelial protein C receptor); (6) the fibrin network (fibrin[ogen], factor XIII, thrombin-activatable fibrinolysis inhibitor); and (7) inhibitors of coagulation (antithrombin, tissue factor pathway inhibitor, protein Z/protein Z-dependent protease inhibitor). Table 113–1 summarizes the major features of the coagulation factors addressed in this chapter. The final sections of this chapter present an overview of the coagulation cascade in which the pathways of hemostasis including the contribution of endothelial cells, blood platelets, and immune cells are described.

Acronyms and Abbreviations

APC, activated protein C; ADP, adenosine diphosphate; AT, antithrombin; C4BP, complement 4b–binding protein; COX, cyclooxygenase; EGF, epidermal growth factor; EPCR, endothelial protein C receptor; ER, endoplasmatic reticulum; ERGIC, ER-Golgi intermediate compartment; Gla, γ-carboxy glutamic acid; GP, glycoprotein; HK, high-molecular-weight kininogen; LMAN1, mannose-binding lectin-1; PAI-1, plasminogen activator inhibitor type 1; PAR, protease-activated receptor; PK, prekallikrein; poly-P, polyphosphate; RVV, Russell’s viper venom; SHBG, sex hormone–binding globulin; TAFI, thrombin-activatable fibrinolysis inhibitor; TFPI, tissue factor pathway inhibitor; UFH, unfractionated heparin; VWD, von Willebrand disease; VWF, von Willebrand factor; ZPI, protein Z–dependent protease inhibitor.

Table 113–1.Characteristics of Coagulation Proteins
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Table 113–1. Characteristics of Coagulation Proteins
...
Protein Plasma Concentration Mr Plasma Half-Life
(μg/mL) (nmol/L) (kDa) (Hours)
ZYMOGENS
 + Gla domain Prothrombin (factor II) 100 1400 72 60
Factor VII 0.5 10 50 3–6
Factor IX 5 90 55 18–24
Factor X 10 170 59 34–40
Protein C 4 65 62 6–8
 – Gla domain Factor XI 5 30 160 60–80
Factor XII 40 500 80 50–70
Prekallikrein 40 490 85 35
Factor XIIIA*† - – 83 –
Factor XIIIB* 7 94 76.5 –
Factor XIII 30 94 320 240
TAFI 4–15 70–275 60 –
COFACTORS
 Soluble Factor V† 5-10 20 330 12–36
Factor VIII 0.2 0.7 300 8–12
VWF varies 10 500–20,000 8–12
Protein S‡ 25 350 75 42
Protein Z§ 2.5 40

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