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P. 1968

1942  Part XII:  Hemostasis and Thrombosis  Chapter 113:  Molecular Biology and Biochemistry of the Coagulation Factors  1943




                    46.  Winship PR, Dragon AC: Identification of haemophilia B patients with mutations in     78.  Ndonwi M, Broze G: Protein S enhances the tissue factor pathway inhibitor inhibi-
                     the two calcium binding domains of factor IX: Importance of a beta-OH Asp 64—Asn   tion of factor Xa but not its inhibition of factor VIIa-tissue factor. J Thromb Haemost
                     change. Br J Haematol 77:102–109, 1991.               6:1044–1046, 2008.
                    47.  Rallapalli PM, Kemball-Cook G, Tuddenham EG, et al: An interactive mutation data-    79.  Han X, Fiehler R, Broze GJ Jr: Characterization of the protein Z-dependent protease
                     base for human coagulation factor IX provides novel insights into the phenotypes and   inhibitor. Blood 96:3049–3055, 2000.
                     genetics of hemophilia B. J Thromb Haemost 11:1329–1340, 2013.   80.  Roehrig S, Straub A, Pohlmann J, et al: Discovery of the novel antithrombotic agent
                    48.  Begbie ME, Mamdani A, Gataiance S, et al: An important role for the activation peptide   5-chloro-N-({(5S)-2-oxo-3- [4-(3-oxomorpholin-4-yl)phenyl]-1,3-oxazolidin-5-yl}methyl)
                     domain in controlling factor IX levels in the blood of haemophilia B mice. Thromb   thiophene- 2-carboxamide (BAY 59-7939): An oral, direct factor Xa inhibitor. J Med
                     Haemost 94:1138–1147, 2005.                           Chem 48:5900–5908, 2005.
                    49.  Bolt G, Bjelke JR, Hermit MB, et al: Hyperglycosylation prolongs the circulation of     81.  Pinto DJ, Orwat MJ, Koch S, et al: Discovery of 1-(4-methoxyphenyl)-7-oxo-6-(4-(2-ox-
                     coagulation factor IX. J Thromb Haemost 10:2397–2398, 2012.  opiperidin-1-yl)phenyl)-4,5,6,7-tetrahydro-1H-pyrazolo[3,4-c]pyridine-3-carbox-
                    50.  Brooks AR, Sim D, Gritzan U, et al: Glycoengineered factor IX variants with improved   amide (apixaban, BMS-562247), a highly potent, selective, efficacious, and orally
                     pharmacokinetics and subcutaneous efficacy. J Thromb Haemost 11:1699–1706, 2013.  bioavailable inhibitor of blood coagulation factor Xa. J Med Chem 50:5339–5356, 2007.
                    51.  Cheung WF, Hamaguchi N, Smith KJ, Stafford DW: The binding of human factor IX to     82.  Furugohri T, Isobe K, Honda Y, et al: DU-176b, a potent and orally active factor Xa inhib-
                     endothelial cells is mediated by residues 3–11. J Biol Chem 267:20529–20531, 1992.  itor: In vitro and in vivo pharmacological profiles. J Thromb Haemost 6:1542–1549, 2008.
                    52.  Cheung WF, van den Born J, Kühn K, et al: Identification of the endothelial cell binding     83.  Perzborn E, Roehrig S, Straub A, et al: The discovery and development of rivaroxaban,
                     site for factor IX. Proc Natl Acad Sci U S A 93:11068–11073, 1996.  an oral, direct factor Xa inhibitor. Nat Rev Drug Discov 10:61–75, 2011.
                    53.  Lenting PJ, ter Maat H, Clijsters PP, et al: Cleavage at arginine 145 in human blood     84.  Scambler PJ, Williamson R: The structural gene for human coagulation factor X is
                     coagulation factor IX converts the zymogen into a factor VIII binding enzyme. J Biol   located on chromosome 13q34. Cytogenet Cell Genet 39:231–233, 1985.
                     Chem 270:14884–14890, 1995.                          85.  de Visser MC, Poort SR, Vos HL, et al: Factor X levels, polymorphisms in the promoter
                    54.  Lindquist PA, Fujikawa K, Davie EW: Activation of bovine factor IX (Christmas factor)   region of factor X, and the risk of venous thrombosis. Thromb Haemost 85:1011–1017, 2001.
                     by factor XIa (activated plasma thromboplastin antecedent) and a protease from Rus-    86.  Mammen EF, Thomas WR, Seegers WH: Activation of purified prothrombin to auto-
                     sell’s viper venom. J Biol Chem 253:1902–1909, 1978.  prothrombin I or autoprothrombin II (platelet cofactor II or autoprothrombin II-A).
                    55.  Zögg T, Brandstetter H: Activation mechanisms of coagulation factor IX. Biol Chem   Thromb Diath Haemorrh 5:218–249, 1960.
                     390:391–400, 2009.                                   87.  Stenflo J: A new vitamin K-dependent protein. J Biol Chem 251:355–363, 1976.
                    56.  Johnson DJD, Langdown J, Huntington JA: Molecular basis of factor IXa recognition by     88.  Foster DC, Yoshitake S, Davie EW: The nucleotide sequence of the gene for human
                     heparin-activated antithrombin revealed by a 1.7-A structure of the ternary complex.   protein C. Proc Natl Acad Sci U S A 82:4673–4677, 1985.
                     Proc Natl Acad Sci U S A 107:645–650, 2010.          89.  Grinnell BW, Walls JD, Gerlitz B: Glycosylation of human protein C affects its secretion,
                    57.  Camerino G, Grzeschik KH, Jaye M, et al: Regional localization on the human X chro-  processing, functional activities, and activation by thrombin. J Biol Chem 266:9778–
                     mosome and polymorphism of the coagulation factor IX gene (hemophilia B locus).   9785, 1991.
                     Proc Natl Acad Sci U S A 81:498–502, 1984.           90.  McClure DB, Walls JD, Grinnell BW: Post-translational processing events in the secre-
                    58.  van Hylckama Vlieg A, van der Linden IK, Bertina RM, Rosendaal FR: High levels of   tion pathway of human protein C, a complex vitamin K-dependent antithrombotic fac-
                     factor IX increase the risk of venous thrombosis. Blood 95:3678–3682, 2000.  tor. J Biol Chem 267:19710–19717, 1992.
                    59.  Simioni P, Tormene D, Tognin G, et al: X-linked thrombophilia with a mutant factor IX     91.  Preston R, Rawley O: Elucidating the role of carbohydrate determinants in regulating
                     (factor IX Padua). N Engl J Med 361:1671–1675, 2009.  hemostasis: Insights and opportunities. Blood 121:3801–3810, 2013.
                    60.  Denson K: Electrophoretic studies of the Prower factor: A blood coagulation factor     92.  Stearns-Kurosawa DJ, Kurosawa S, Mollica JS, Ferrell GL, Esmon CT: The endothelial
                     which differs from factor VII. Br J Haematol 4:313–325, 1957.  cell protein C receptor augments protein C activation by the thrombin-thrombomod-
                    61.  Telfer TP, Denson KW, Wright DR: A new coagulation defect. Br J Haematol 2:308–316,   ulin complex. Proc Natl Acad Sci U S A 93:10212–10216, 1996.
                     1956.                                                93.  Shen L, Dahlback B: Factor V and protein S as synergistic cofactors to activated protein
                    62.  Hougie C, Barrow EM, Graham JB: Stuart clotting defect. I. Segregation of an hered-  C in degradation of factor VIIIa. J Biol Chem 269:18735–18738, 1994.
                     itary hemorrhagic state from the heterogeneous group heretofore called stable factor     94.  Oliver JA, Monroe DM, Church FC, et al: Activated protein C cleaves factor Va more
                     (SPCA, proconvertin, factor VII) deficiency. J Clin Invest 36:485–496, 1957.  efficiently on endothelium than on platelet surfaces. Blood 100:539–546, 2002.
                    63.  Gueguen P, Cherel G, Badirou I, et al: Two residues in the activation peptide domain     95.  Slungaard A, Fernandez JA, Griffin JH, et al: Platelet factor 4 enhances generation of
                     contribute to the half-life of factor X in vivo. J Thromb Haemost 8:1651–1653, 2010.  activated protein C in vitro and in vivo. Blood 102:146–151, 2003.
                    64.  Rudolph AE, Mullane MP, Porche-Sorbet R, et al: The role of the factor X activation     96.  Bouwens EA, Stavenuiter F, Mosnier LO: Mechanisms of anticoagulant and cytoprotec-
                     peptide: A deletion mutagenesis approach. Thromb Haemost 88:756–762, 2002.  tive actions of the protein C pathway. J Thromb Haemost 11 Suppl 1:242–253, 2013.
                    65.  Yang L, Manithody C, Rezaie AR: Functional role of O-linked and N-linked glycosyla-    97.  Foster DC, Yoshitake S, Davie EW: The nucleotide sequence of the gene for human
                     tion sites present on the activation peptide of factor X. J Thromb Haemost 7:1696–1702,   protein C. Proc Natl Acad Sci U S A 82:4673–4677, 1985.
                     2009.                                                98.  Branson HE, Katz J, Marble R, Griffin JH: Inherited protein C deficiency and cou-
                    66.  Rao LV, Rapaport SI: Activation of factor VII bound to tissue factor: A key early step in the   marin-responsive chronic relapsing purpura fulminans in a newborn infant. Lancet
                     tissue factor pathway of blood coagulation. Proc Natl Acad Sci U S A 85:6687–6691, 1988.  2:1165–1168, 1983.
                    67.  Monkovic DD, Tracy PB: Activation of human factor V by factor Xa and thrombin.     99.  Reitsma  PH:  Protein  C  deficiency:  From  gene  defects  to  disease.  Thromb Haemost
                     Biochemistry 29:1118–1128, 1990.                      78:344–350, 1997.
                    68.  Neuenschwander PF, Jesty J: Thrombin-activated and factor Xa-activated human factor     100. Lijfering WM, Christiansen SC, Rosendaal FR, Cannegieter SC: Contribution of high
                     VIII: Differences in cofactor activity and decay rate. Arch Biochem Biophys 296:426–  factor VIII, IX and XI to the risk of recurrent venous thrombosis in factor V Leiden
                     434, 1992.                                            carriers. J Thromb Haemost 7:1944–1946, 2009.
                    69.  Borensztajn K, Peppelenbosch MP, Spek CA: Factor Xa: At the crossroads between     101. Spek CA, Koster T, Rosendaal FR, et al: Genotypic variation in the promoter region
                     coagulation and signaling in physiology and disease. Trends Mol Med 14:429–440, 2008.  of the protein C gene is associated with plasma protein C levels and thrombotic risk.
                    70.  Jesty J, Spencer AK, Nemerson Y: The mechanism of activation of factor X. Kinetic con-  Arterioscler Thromb Vasc Biol 15:214–218, 1995.
                     trol of alternative pathways leading to the formation of activated factor X. J Biol Chem     102. Davidson CJ, Tuddenham EG, McVey JH: 450 Million years of hemostasis. J Thromb
                     249:5614–5622, 1974.                                  Haemost 1:1487–1494, 2003.
                    71.  Fujikawa K, Titani K, Davie EW: Activation of bovine factor X (Stuart factor): Conver-    103. Zheng C, Zhang B: Combined deficiency of coagulation factors V and VIII: An update.
                     sion of factor Xaalpha to factor Xabeta. Proc Natl Acad Sci U S A 72:3359–3363, 1975.  Semin Thromb Hemost 39:613–620, 2013.
                    72.  Pryzdial EL, Kessler GE: Kinetics of blood coagulation factor Xaalpha autoproteolytic     104. Owren PA: The coagulation of blood: Investigations on a new clotting factor. Acta Med
                     conversion to factor Xabeta. Effect on inhibition by antithrombin, prothrombinase   Scand 128 (Suppl 194), 1947.
                     assembly, and enzyme activity. J Biol Chem 271:16621–16626, 1996.    105. Owren PA: Parahaemophilia. Haemorrhagic diathesis due to absence of a previously
                    73.  Pryzdial EL, Kessler GE: Autoproteolysis or plasmin-mediated cleavage of factor   unknown clotting factor. Lancet 1:446–448, 1947.
                     Xaalpha  exposes  a  plasminogen  binding  site  and  inhibits  coagulation.  J Biol Chem     106. Stormorken H: The discovery of factor V: A tricky clotting factor. J Thromb Haemost
                     271:16614–16620, 1996.                                1:206–213, 2003.
                    74.  Grundy JE, Lavigne N, Hirama T, et al: Binding of plasminogen and tissue plasminogen     107. Camire RM, Pollak ES, Kaushansky K, Tracy PB: Secretable human platelet-derived
                     activator to plasmin-modulated factor X and factor Xa. Biochemistry 40:6293–6302,   factor V originates from the plasma pool. Blood 92:3035–3041, 1998.
                     2001.                                                108. Thomassen MC, Castoldi E, Tans G, et al: Endogenous factor V synthesis in
                    75.  Talbot K, Meixner SC, Pryzdial ELG: Enhanced fibrinolysis by proteolysed coagulation   megakaryocytes contributes negligibly to the platelet factor V pool. Haematologica
                     factor Xa. Biochim Biophys Acta 1804:723–730, 2010.   88:1150–1156, 2003.
                    76.  Johnson DJD, Li W, Adams TE, Huntington Ja. Antithrombin-S195A factor Xa-hep-    109. Bouchard BA, Williams JL, Meisler NT, et al: Endocytosis of plasma-derived factor V
                     arin structure reveals the allosteric mechanism of antithrombin activation. EMBO J   by megakaryocytes occurs via a clathrin-dependent, specific membrane binding event.
                     25:2029–2037, 2006.                                   J Thromb Haemost 3:541–551, 2005.
                    77.  Hackeng TM, Seré KM, Tans G, Rosing J: Protein S stimulates inhibition of the tis-    110. Gould WR, Silveira JR, Tracy PB: Unique in vivo modifications of coagulation factor V
                     sue factor pathway by tissue factor pathway inhibitor. Proc Natl Acad Sci U S A 103:   produce a physically and functionally distinct platelet-derived cofactor: Characteriza-
                     3106–3111, 2006.                                      tion of purified platelet-derived factor V/Va. J Biol Chem 279:2383–2393, 2004.







          Kaushansky_chapter 113_p1915-1948.indd   1943                                                                 9/21/15   2:41 PM
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