Formation of Fibrin Clot (Clotting Cascade) (Homo sapiens)

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1, 254-577477, 791328, 3067-722365, 6617862236-39271376, 80, 8167, 68, 71, 732624, 28, 2935, 38, 45, 4632-358617, 208558, 62-6482836, 82658-61863140-4417828623-2576-788434, 47-5375cytoplasmprotein C heavy chain activation peptidefactor VIIaActivated thrombin (factor IIa)thrombin:cleaved antithrombin IIIfactor XaTF:F7afactor XIINH4+Platelet Factor 4activated protein Cfactor VIIIakallikrein:C1InhkininogenTFPIfactor XIIaBradykininthrombin:cleaved antithrombin III:heparinprekallikrein:kininogen:C1q binding protein tetramerC1InhTFPI:TF:F7a:factor Xafactor VIIIactivated kininogen:C1q binding protein tetramerfactor XIIICalciumfactor Xfactor X activation peptideHeparinCalciumantithrombin IIIkininogen:C1q binding protein tetramerprothrombin (factor II)factor IXafibrin multimer, crosslinkedfibrinopeptide Avon Willibrand factor multimeralpha2-macroglobulinfactor IX activation peptidefactor Vifibrinopeptide BCalciumTF:F7factor XI:GPIb:GPIX:GPV complexfactor V activation peptideVa:Xa complex (prothrombinase)factor XIII B chainfactor XIfactor VIIprekallikreinGPIb:GPIX:GPV complexfactor XIII A chain activation peptidefactor Xaactivated protein Cthrombomodulinfactor VIII:von Willebrand factor multimeractivated thrombin:thrombomodulinfactor XIII cleaved tetramerfactor VIIIa:factor IXaC1q binding protein tetramerZn++protein SCalciumfactor Vafactor VIIIa B A3 acidic polypeptidekallikrein:alpha2-macroglobulinfibrin monomerFibrinogensequestered tissue factorkallikreinprotein Cfactor XIa:GPIb:GPIX:GPV complexkininogentissue factorthrombin:antithrombin III:heparinfactor XIIIathrombin activation peptidefactor Vprolylcarboxypeptidase dimerfactor XIIa:C1Inhfibrin multimerfactor IXantithrombin III:heparinkallikrein:kininogen:C1q binding protein tetramer810, 12155, 6618, 1910-125, 6171418, 198718-208, 93, 41318-2016215133, 4


The formation of a fibrin clot at the site of an injury to the wall of a normal blood vessel is an essential part of the process to stop blood loss after vascular injury. The reactions that lead to fibrin clot formation are commonly described as a cascade, in which the product of each step is an enzyme or cofactor needed for following reactions to proceed efficiently. The entire clotting cascade can be divided into three portions, the extrinsic pathway, the intrinsic pathway, and the common pathway. The extrinsic pathway begins with the release of tissue factor at the site of vascular injury and leads to the activation of factor X. The intrinsic pathway provides an alternative mechanism for activation of factor X, starting from the activation of factor XII. The common pathway consists of the steps linking the activation of factor X to the formation of a multimeric, cross-linked fibrin clot. Each of these pathways includes not only a cascade of events that generate the catalytic activities needed for clot formation, but also numerous positive and negative regulatory events.

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  1. Baglia FA, Badellino KO, Li CQ, Lopez JA, Walsh PN.; ''Factor XI binding to the platelet glycoprotein Ib-IX-V complex promotes factor XI activation by thrombin.''; PubMed Europe PMC Scholia
  2. Eaton D, Rodriguez H, Vehar GA.; ''Proteolytic processing of human factor VIII. Correlation of specific cleavages by thrombin, factor Xa, and activated protein C with activation and inactivation of factor VIII coagulant activity.''; PubMed Europe PMC Scholia
  3. Orfeo T, Brufatto N, Nesheim ME, Xu H, Butenas S, Mann KG.; ''The factor V activation paradox.''; PubMed Europe PMC Scholia
  4. Schmaier AH.; ''The physiologic basis of assembly and activation of the plasma kallikrein/kinin system.''; PubMed Europe PMC Scholia
  5. Naito K, Fujikawa K.; ''Activation of human blood coagulation factor XI independent of factor XII. Factor XI is activated by thrombin and factor XIa in the presence of negatively charged surfaces.''; PubMed Europe PMC Scholia
  6. Gilbert GE, Furie BC, Furie B.; ''Binding of human factor VIII to phospholipid vesicles.''; PubMed Europe PMC Scholia
  7. Leytus SP, Chung DW, Kisiel W, Kurachi K, Davie EW.; ''Characterization of a cDNA coding for human factor X.''; PubMed Europe PMC Scholia
  8. Church FC, Noyes CM, Griffith MJ.; ''Inhibition of chymotrypsin by heparin cofactor II.''; PubMed Europe PMC Scholia
  9. Banner DW, D'Arcy A, Chène C, Winkler FK, Guha A, Konigsberg WH, Nemerson Y, Kirchhofer D.; ''The crystal structure of the complex of blood coagulation factor VIIa with soluble tissue factor.''; PubMed Europe PMC Scholia
  10. Rapaport SI, Rao LV.; ''The tissue factor pathway: how it has become a "prima ballerina".''; PubMed Europe PMC Scholia
  11. McMullen BA, Fujikawa K, Davie EW.; ''Location of the disulfide bonds in human coagulation factor XI: the presence of tandem apple domains.''; PubMed Europe PMC Scholia
  12. McMullen BA, Fujikawa K, Kisiel W.; ''The occurrence of beta-hydroxyaspartic acid in the vitamin K-dependent blood coagulation zymogens.''; PubMed Europe PMC Scholia
  13. Komiyama Y, Pedersen AH, Kisiel W.; ''Proteolytic activation of human factors IX and X by recombinant human factor VIIa: effects of calcium, phospholipids, and tissue factor.''; PubMed Europe PMC Scholia
  14. Ratnoff OD, Pensky J, Ogston D, Naff GB.; ''The inhibition of plasmin, plasma kallikrein, plasma permeability factor, and the C'1r subcomponent of the first component of complement by serum C'1 esterase inhibitor.''; PubMed Europe PMC Scholia
  15. Walz DA, Hewett-Emmett D, Seegers WH.; ''Amino acid sequence of human prothrombin fragments 1 and 2.''; PubMed Europe PMC Scholia
  16. Di Scipio RG, Hermodson MA, Davie EW.; ''Activation of human factor X (Stuart factor) by a protease from Russell's viper venom.''; PubMed Europe PMC Scholia
  17. Vlot AJ, Koppelman SJ, van den Berg MH, Bouma BN, Sixma JJ.; ''The affinity and stoichiometry of binding of human factor VIII to von Willebrand factor.''; PubMed Europe PMC Scholia
  18. Wienen W, Stassen JM, Priepke H, Ries UJ, Hauel N.; ''In-vitro profile and ex-vivo anticoagulant activity of the direct thrombin inhibitor dabigatran and its orally active prodrug, dabigatran etexilate.''; PubMed Europe PMC Scholia
  19. Harpel PC, Lewin MF, Kaplan AP.; ''Distribution of plasma kallikrein between C-1 inactivator and alpha 2-macroglobulin in plasma utilizing a new assay for alpha 2-macroglobulin-kallikrein complexes.''; PubMed Europe PMC Scholia
  20. Davie EW, Fujikawa K, Kisiel W.; ''The coagulation cascade: initiation, maintenance, and regulation.''; PubMed Europe PMC Scholia
  21. Bondarenko M, Curti C, Montana M, Rathelot P, Vanelle P.; ''Efficacy and toxicity of factor Xa inhibitors.''; PubMed Europe PMC Scholia
  22. Hill-Eubanks DC, Parker CG, Lollar P.; ''Differential proteolytic activation of factor VIII-von Willebrand factor complex by thrombin.''; PubMed Europe PMC Scholia
  23. Bhanwra S, Ahluwalia K.; ''The new factor Xa inhibitor: Apixaban.''; PubMed Europe PMC Scholia
  24. Modderman PW, Admiraal LG, Sonnenberg A, von dem Borne AE.; ''Glycoproteins V and Ib-IX form a noncovalent complex in the platelet membrane.''; PubMed Europe PMC Scholia
  25. Suzuki K, Nishioka J, Kusumoto H, Hashimoto S.; ''Mechanism of inhibition of activated protein C by protein C inhibitor.''; PubMed Europe PMC Scholia
  26. Wilcox JN, Smith KM, Schwartz SM, Gordon D.; ''Localization of tissue factor in the normal vessel wall and in the atherosclerotic plaque.''; PubMed Europe PMC Scholia
  27. Kane WH, Davie EW.; ''Cloning of a cDNA coding for human factor V, a blood coagulation factor homologous to factor VIII and ceruloplasmin.''; PubMed Europe PMC Scholia
  28. Gilbert GE, Arena AA.; ''Activation of the factor VIIIa-factor IXa enzyme complex of blood coagulation by membranes containing phosphatidyl-L-serine.''; PubMed Europe PMC Scholia
  29. Pixley RA, Schapira M, Colman RW.; ''The regulation of human factor XIIa by plasma proteinase inhibitors.''; PubMed Europe PMC Scholia
  30. Laudano AP, Doolittle RF.; ''Studies on synthetic peptides that bind to fibrinogen and prevent fibrin polymerization. Structural requirements, number of binding sites, and species differences.''; PubMed Europe PMC Scholia
  31. Minor C, Tellor KB, Armbruster AL.; ''Edoxaban, a Novel Oral Factor Xa Inhibitor.''; PubMed Europe PMC Scholia
  32. Pieters J, Lindhout T, Hemker HC.; ''In situ-generated thrombin is the only enzyme that effectively activates factor VIII and factor V in thromboplastin-activated plasma.''; PubMed Europe PMC Scholia
  33. Di Scipio RG, Hermodson MA, Yates SG, Davie EW.; ''A comparison of human prothrombin, factor IX (Christmas factor), factor X (Stuart factor), and protein S.''; PubMed Europe PMC Scholia
  34. Kurachi K, Davie EW.; ''Activation of human factor XI (plasma thromboplastin antecedent) by factor XIIa (activated Hageman factor).''; PubMed Europe PMC Scholia
  35. Taylor FB, Peer GT, Lockhart MS, Ferrell G, Esmon CT.; ''Endothelial cell protein C receptor plays an important role in protein C activation in vivo.''; PubMed Europe PMC Scholia
  36. Wen DZ, Dittman WA, Ye RD, Deaven LL, Majerus PW, Sadler JE.; ''Human thrombomodulin: complete cDNA sequence and chromosome localization of the gene.''; PubMed Europe PMC Scholia
  37. Graetz TJ, Tellor BR, Smith JR, Avidan MS.; ''Desirudin: a review of the pharmacology and clinical application for the prevention of deep vein thrombosis.''; PubMed Europe PMC Scholia
  38. Foster DC, Sprecher CA, Holly RD, Gambee JE, Walker KM, Kumar AA.; ''Endoproteolytic processing of the dibasic cleavage site in the human protein C precursor in transfected mammalian cells: effects of sequence alterations on efficiency of cleavage.''; PubMed Europe PMC Scholia
  39. Fujikawa K, McMullen BA.; ''Amino acid sequence of human beta-factor XIIa.''; PubMed Europe PMC Scholia
  40. Schreiber AD, Kaplan AP, Austen KF.; ''Inhibition by C1INH of Hagemann factor fragment activation of coagulation, fibrinolysis, and kinin generation.''; PubMed Europe PMC Scholia
  41. Yegneswaran S, Smirnov MD, Safa O, Esmon NL, Esmon CT, Johnson AE.; ''Relocating the active site of activated protein C eliminates the need for its protein S cofactor. A fluorescence resonance energy transfer study.''; PubMed Europe PMC Scholia
  42. Broze GJ, Girard TJ, Novotny WF.; ''Regulation of coagulation by a multivalent Kunitz-type inhibitor.''; PubMed Europe PMC Scholia
  43. Mertens K, Cupers R, Van Wijngaarden A, Bertina RM.; ''Binding of human blood-coagulation Factors IXa and X to phospholipid membranes.''; PubMed Europe PMC Scholia
  44. Chiu AT, Mousa SA, Pease LJ, Roscoe WA, Bozarth JM, Reilly TM, Smith RD, Timmermans PB.; ''Inhibition of the thrombin-platelet reactions by DuP 714.''; PubMed Europe PMC Scholia
  45. Silverberg M, Dunn JT, Garen L, Kaplan AP.; ''Autoactivation of human Hageman factor. Demonstration utilizing a synthetic substrate.''; PubMed Europe PMC Scholia
  46. Li W, Huntington JA.; ''Crystal structures of protease nexin-1 in complex with heparin and thrombin suggest a 2-step recognition mechanism.''; PubMed Europe PMC Scholia
  47. Griffin JH, Cochrane CG.; ''Mechanisms for the involvement of high molecular weight kininogen in surface-dependent reactions of Hageman factor.''; PubMed Europe PMC Scholia
  48. Lollar P, Hill-Eubanks DC, Parker CG.; ''Association of the factor VIII light chain with von Willebrand factor.''; PubMed Europe PMC Scholia
  49. Rosing J, Hoekema L, Nicolaes GA, Thomassen MC, Hemker HC, Varadi K, Schwarz HP, Tans G.; ''Effects of protein S and factor Xa on peptide bond cleavages during inactivation of factor Va and factor VaR506Q by activated protein C.''; PubMed Europe PMC Scholia
  50. Lawson JH, Mann KG.; ''Cooperative activation of human factor IX by the human extrinsic pathway of blood coagulation.''; PubMed Europe PMC Scholia
  51. Gailani D, Broze GJ.; ''Factor XII-independent activation of factor XI in plasma: effects of sulfatides on tissue factor-induced coagulation.''; PubMed Europe PMC Scholia
  52. Broze GJ.; ''Binding of human factor VII and VIIa to monocytes.''; PubMed Europe PMC Scholia
  53. Vehar GA, Keyt B, Eaton D, Rodriguez H, O'Brien DP, Rotblat F, Oppermann H, Keck R, Wood WI, Harkins RN, Tuddenham EG, Lawn RM, Capon DJ.; ''Structure of human factor VIII.''; PubMed Europe PMC Scholia
  54. Kurosawa S, Stearns-Kurosawa DJ, Hidari N, Esmon CT.; ''Identification of functional endothelial protein C receptor in human plasma.''; PubMed Europe PMC Scholia
  55. Nemerson Y.; ''Tissue factor and hemostasis.''; PubMed Europe PMC Scholia
  56. Lewis SD, Janus TJ, Lorand L, Shafer JA.; ''Regulation of formation of factor XIIIa by its fibrin substrates.''; PubMed Europe PMC Scholia
  57. Zhang P, Huang W, Wang L, Bao L, Jia ZJ, Bauer SM, Goldman EA, Probst GD, Song Y, Su T, Fan J, Wu Y, Li W, Woolfrey J, Sinha U, Wong PW, Edwards ST, Arfsten AE, Clizbe LA, Kanter J, Pandey A, Park G, Hutchaleelaha A, Lambing JL, Hollenbach SJ, Scarborough RM, Zhu BY.; ''Discovery of betrixaban (PRT054021), N-(5-chloropyridin-2-yl)-2-(4-(N,N-dimethylcarbamimidoyl)benzamido)-5-methoxybenzamide, a highly potent, selective, and orally efficacious factor Xa inhibitor.''; PubMed Europe PMC Scholia
  58. Regan LM, Lamphear BJ, Huggins CF, Walker FJ, Fay PJ.; ''Factor IXa protects factor VIIIa from activated protein C. Factor IXa inhibits activated protein C-catalyzed cleavage of factor VIIIa at Arg562.''; PubMed Europe PMC Scholia
  59. Osterud B, Bouma BN, Griffin JH.; ''Human blood coagulation factor IX. Purification, properties, and mechanism of activation by activated factor XI.''; PubMed Europe PMC Scholia
  60. Hagen FS, Gray CL, O'Hara P, Grant FJ, Saari GC, Woodbury RG, Hart CE, Insley M, Kisiel W, Kurachi K.; ''Characterization of a cDNA coding for human factor VII.''; PubMed Europe PMC Scholia
  61. Lin Y, Harris RB, Yan W, McCrae KR, Zhang H, Colman RW.; ''High molecular weight kininogen peptides inhibit the formation of kallikrein on endothelial cell surfaces and subsequent urokinase-dependent plasmin formation.''; PubMed Europe PMC Scholia
  62. Gustafsson D, Antonsson T, Bylund R, Eriksson U, Gyzander E, Nilsson I, Elg M, Mattsson C, Deinum J, Pehrsson S, Karlsson O, Nilsson A, Sörensen H.; ''Effects of melagatran, a new low-molecular-weight thrombin inhibitor, on thrombin and fibrinolytic enzymes.''; PubMed Europe PMC Scholia
  63. Sottrup-Jensen L, Stepanik TM, Kristensen T, Wierzbicki DM, Jones CM, Lønblad PB, Magnusson S, Petersen TE.; ''Primary structure of human alpha 2-macroglobulin. V. The complete structure.''; PubMed Europe PMC Scholia
  64. Saenko EL, Scandella D, Yakhyaev AV, Greco NJ.; ''Activation of factor VIII by thrombin increases its affinity for binding to synthetic phospholipid membranes and activated platelets.''; PubMed Europe PMC Scholia
  65. Hursting MJ, Alford KL, Becker JC, Brooks RL, Joffrion JL, Knappenberger GD, Kogan PW, Kogan TP, McKinney AA, Schwarz RP.; ''Novastan (brand of argatroban): a small-molecule, direct thrombin inhibitor.''; PubMed Europe PMC Scholia
  66. Baglia FA, Walsh PN.; ''Thrombin-mediated feedback activation of factor XI on the activated platelet surface is preferred over contact activation by factor XIIa or factor XIa.''; PubMed Europe PMC Scholia
  67. Foster D, Davie EW.; ''Characterization of a cDNA coding for human protein C.''; PubMed Europe PMC Scholia
  68. Roehrig S, Straub A, Pohlmann J, Lampe T, Pernerstorfer J, Schlemmer KH, Reinemer P, Perzborn E.; ''Discovery of the novel antithrombotic agent 5-chloro-N-({(5S)-2-oxo-3- [4-(3-oxomorpholin-4-yl)phenyl]-1,3-oxazolidin-5-yl}methyl)thiophene- 2-carboxamide (BAY 59-7939): an oral, direct factor Xa inhibitor.''; PubMed Europe PMC Scholia
  69. Tan F, Morris PW, Skidgel RA, Erdös EG.; ''Sequencing and cloning of human prolylcarboxypeptidase (angiotensinase C). Similarity to both serine carboxypeptidase and prolylendopeptidase families.''; PubMed Europe PMC Scholia
  70. Oliver JA, Monroe DM, Roberts HR, Hoffman M.; ''Thrombin activates factor XI on activated platelets in the absence of factor XII.''; PubMed Europe PMC Scholia
  71. Mann KG, Jenny RJ, Krishnaswamy S.; ''Cofactor proteins in the assembly and expression of blood clotting enzyme complexes.''; PubMed Europe PMC Scholia
  72. Kisiel W.; ''Human plasma protein C: isolation, characterization, and mechanism of activation by alpha-thrombin.''; PubMed Europe PMC Scholia
  73. Stangier J, Rathgen K, Stähle H, Gansser D, Roth W.; ''The pharmacokinetics, pharmacodynamics and tolerability of dabigatran etexilate, a new oral direct thrombin inhibitor, in healthy male subjects.''; PubMed Europe PMC Scholia
  74. Weitz JI, Hudoba M, Massel D, Maraganore J, Hirsh J.; ''Clot-bound thrombin is protected from inhibition by heparin-antithrombin III but is susceptible to inactivation by antithrombin III-independent inhibitors.''; PubMed Europe PMC Scholia
  75. Kane WH, Ichinose A, Hagen FS, Davie EW.; ''Cloning of cDNAs coding for the heavy chain region and connecting region of human factor V, a blood coagulation factor with four types of internal repeats.''; PubMed Europe PMC Scholia
  76. Ichinose A, Hendrickson LE, Fujikawa K, Davie EW.; ''Amino acid sequence of the a subunit of human factor XIII.''; PubMed Europe PMC Scholia
  77. Joseph K, Ghebrehiwet B, Peerschke EI, Reid KB, Kaplan AP.; ''Identification of the zinc-dependent endothelial cell binding protein for high molecular weight kininogen and factor XII: identity with the receptor that binds to the globular "heads" of C1q (gC1q-R).''; PubMed Europe PMC Scholia
  78. Ghebrehiwet B, Lim BL, Peerschke EI, Willis AC, Reid KB.; ''Isolation, cDNA cloning, and overexpression of a 33-kD cell surface glycoprotein that binds to the globular "heads" of C1q.''; PubMed Europe PMC Scholia
  79. Kurosawa S, Esmon CT, Stearns-Kurosawa DJ.; ''The soluble endothelial protein C receptor binds to activated neutrophils: involvement of proteinase-3 and CD11b/CD18.''; PubMed Europe PMC Scholia
  80. Mushunje A, Zhou A, Carrell RW, Huntington JA.; ''Heparin-induced substrate behavior of antithrombin Cambridge II.''; PubMed Europe PMC Scholia
  81. Titani K, Kumar S, Takio K, Ericsson LH, Wade RD, Ashida K, Walsh KA, Chopek MW, Sadler JE, Fujikawa K.; ''Amino acid sequence of human von Willebrand factor.''; PubMed Europe PMC Scholia
  82. Bach R, Gentry R, Nemerson Y.; ''Factor VII binding to tissue factor in reconstituted phospholipid vesicles: induction of cooperativity by phosphatidylserine.''; PubMed Europe PMC Scholia
  83. Chung DW, Fujikawa K, McMullen BA, Davie EW.; ''Human plasma prekallikrein, a zymogen to a serine protease that contains four tandem repeats.''; PubMed Europe PMC Scholia
  84. Yoshitake S, Schach BG, Foster DC, Davie EW, Kurachi K.; ''Nucleotide sequence of the gene for human factor IX (antihemophilic factor B).''; PubMed Europe PMC Scholia
  85. Moreira CR, Schmaier AH, Mahdi F, da Motta G, Nader HB, Shariat-Madar Z.; ''Identification of prolylcarboxypeptidase as the cell matrix-associated prekallikrein activator.''; PubMed Europe PMC Scholia
  86. Mahdi F, Shariat-Madar Z, Schmaier AH.; ''The relative priority of prekallikrein and factors XI/XIa assembly on cultured endothelial cells.''; PubMed Europe PMC Scholia
  87. Shariat-Madar Z, Mahdi F, Schmaier AH.; ''Identification and characterization of prolylcarboxypeptidase as an endothelial cell prekallikrein activator.''; PubMed Europe PMC Scholia
  88. Kerbiriou DM, Griffin JH.; ''Human high molecular weight kininogen. Studies of structure-function relationships and of proteolysis of the molecule occurring during contact activation of plasma.''; PubMed Europe PMC Scholia
  89. Griffin JH, Fernández JA, Gale AJ, Mosnier LO.; ''Activated protein C.''; PubMed Europe PMC Scholia
  90. McMullen BA, Fujikawa K.; ''Amino acid sequence of the heavy chain of human alpha-factor XIIa (activated Hageman factor).''; PubMed Europe PMC Scholia
  91. Riewald M, Petrovan RJ, Donner A, Mueller BM, Ruf W.; ''Activation of endothelial cell protease activated receptor 1 by the protein C pathway.''; PubMed Europe PMC Scholia
  92. Motta G, Rojkjaer R, Hasan AA, Cines DB, Schmaier AH.; ''High molecular weight kininogen regulates prekallikrein assembly and activation on endothelial cells: a novel mechanism for contact activation.''; PubMed Europe PMC Scholia
  93. Degen SJ, Davie EW.; ''Nucleotide sequence of the gene for human prothrombin.''; PubMed Europe PMC Scholia
  94. Bock SC, Wion KL, Vehar GA, Lawn RM.; ''Cloning and expression of the cDNA for human antithrombin III.''; PubMed Europe PMC Scholia
  95. Mann KG, Kalafatis M.; ''Factor V: a combination of Dr Jekyll and Mr Hyde.''; PubMed Europe PMC Scholia
  96. Shariat-Madar Z, Mahdi F, Schmaier AH.; ''Recombinant prolylcarboxypeptidase activates plasma prekallikrein.''; PubMed Europe PMC Scholia
  97. Bock SC, Skriver K, Nielsen E, Thøgersen HC, Wiman B, Donaldson VH, Eddy RL, Marrinan J, Radziejewska E, Huber R.; ''Human C1 inhibitor: primary structure, cDNA cloning, and chromosomal localization.''; PubMed Europe PMC Scholia
  98. Odya CE, Marinkovic DV, Hammon KJ, Stewart TA, Erdös EG.; ''Purification and properties of prolylcarboxypeptidase (angiotensinase C) from human kidney.''; PubMed Europe PMC Scholia
  99. Gladwell TD.; ''Bivalirudin: a direct thrombin inhibitor.''; PubMed Europe PMC Scholia
  100. Ichinose A, McMullen BA, Fujikawa K, Davie EW.; ''Amino acid sequence of the b subunit of human factor XIII, a protein composed of ten repetitive segments.''; PubMed Europe PMC Scholia
  101. Esmon CT.; ''The roles of protein C and thrombomodulin in the regulation of blood coagulation.''; PubMed Europe PMC Scholia
  102. Gailani D, Ho D, Sun MF, Cheng Q, Walsh PN.; ''Model for a factor IX activation complex on blood platelets: dimeric conformation of factor XIa is essential.''; PubMed Europe PMC Scholia
  103. DiScipio RG, Davie EW.; ''Characterization of protein S, a gamma-carboxyglutamic acid containing protein from bovine and human plasma.''; PubMed Europe PMC Scholia
  104. Ni F, Konishi Y, Frazier RB, Scheraga HA, Lord ST.; ''High-resolution NMR studies of fibrinogen-like peptides in solution: interaction of thrombin with residues 1-23 of the A alpha chain of human fibrinogen.''; PubMed Europe PMC Scholia
  105. Bouma BN, Griffin JH.; ''Human blood coagulation factor XI. Purification, properties, and mechanism of activation by activated factor XII.''; PubMed Europe PMC Scholia
  106. Greengard JS, Heeb MJ, Ersdal E, Walsh PN, Griffin JH.; ''Binding of coagulation factor XI to washed human platelets.''; PubMed Europe PMC Scholia
  107. Joseph K, Shibayama Y, Ghebrehiwet B, Kaplan AP.; ''Factor XII-dependent contact activation on endothelial cells and binding proteins gC1qR and cytokeratin 1.''; PubMed Europe PMC Scholia
  108. Rawala-Sheikh R, Ahmad SS, Ashby B, Walsh PN.; ''Kinetics of coagulation factor X activation by platelet-bound factor IXa.''; PubMed Europe PMC Scholia
  109. Mann KG, Butenas S, Brummel K.; ''The dynamics of thrombin formation.''; PubMed Europe PMC Scholia
  110. Kellermann J, Lottspeich F, Henschen A, Müller-Esterl W.; ''Completion of the primary structure of human high-molecular-mass kininogen. The amino acid sequence of the entire heavy chain and evidence for its evolution by gene triplication.''; PubMed Europe PMC Scholia
  111. Meier HL, Pierce JV, Colman RW, Kaplan AP.; ''Activation and function of human Hageman factor. The role of high molecular weight kininogen and prekallikrein.''; PubMed Europe PMC Scholia
  112. Butenas S, Mann KG.; ''Kinetics of human factor VII activation.''; PubMed Europe PMC Scholia
  113. Thim L, Bjoern S, Christensen M, Nicolaisen EM, Lund-Hansen T, Pedersen AH, Hedner U.; ''Amino acid sequence and posttranslational modifications of human factor VIIa from plasma and transfected baby hamster kidney cells.''; PubMed Europe PMC Scholia
  114. Mahdi F, Madar ZS, Figueroa CD, Schmaier AH.; ''Factor XII interacts with the multiprotein assembly of urokinase plasminogen activator receptor, gC1qR, and cytokeratin 1 on endothelial cell membranes.''; PubMed Europe PMC Scholia
  115. Lottspeich F, Kellermann J, Henschen A, Foertsch B, Müller-Esterl W.; ''The amino acid sequence of the light chain of human high-molecular-mass kininogen.''; PubMed Europe PMC Scholia
  116. Shrimpton CN, Borthakur G, Larrucea S, Cruz MA, Dong JF, López JA.; ''Localization of the adhesion receptor glycoprotein Ib-IX-V complex to lipid rafts is required for platelet adhesion and activation.''; PubMed Europe PMC Scholia
  117. Butkowski RJ, Elion J, Downing MR, Mann KG.; ''Primary structure of human prethrombin 2 and alpha-thrombin.''; PubMed Europe PMC Scholia
  118. McMullen BA, Fujikawa K, Kisiel W, Sasagawa T, Howald WN, Kwa EY, Weinstein B.; ''Complete amino acid sequence of the light chain of human blood coagulation factor X: evidence for identification of residue 63 as beta-hydroxyaspartic acid.''; PubMed Europe PMC Scholia


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External references


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NameTypeDatabase referenceComment
Activated thrombin

(factor IIa)

ComplexREACT_3298 (Reactome)
Bradykinin ProteinP01042 (UniProt)
C1Inh ProteinP05155 (UniProt)
C1q binding protein


ComplexREACT_2937 (Reactome)
Calcium Metabolite29108 (ChEBI)
Fibrinogen ComplexREACT_4095 (Reactome) Fibrinogen is a hexamer, containing two fibrinogen alpha chains, two fibrinogen beta chains, and two fibrinogen gamma chains, held together by disulfide bonds.


ComplexREACT_2987 (Reactome)
Heparin UnknownREACT_4737 (Reactome)
NH4+ Metabolite28938 (ChEBI)
Platelet Factor 4 UnknownREACT_12205 (Reactome)
TF:F7 ComplexREACT_4908 (Reactome)
TF:F7a ComplexREACT_4532 (Reactome)
TFPI ProteinP10646 (UniProt)
TFPI:TF:F7a:factor Xa ComplexREACT_2685 (Reactome)
Va:Xa complex


ComplexREACT_5441 (Reactome)
Zn++ Metabolite29105 (ChEBI)

thrombin: thrombomodulin

ComplexREACT_5904 (Reactome)
activated kininogen:

C1q binding protein tetramer

ComplexREACT_3121 (Reactome)
activated protein C ComplexREACT_2599 (Reactome)
activated protein C ComplexREACT_5044 (Reactome)


ComplexREACT_3449 (Reactome)


ProteinP01008 (UniProt)
antithrombin III:


ComplexREACT_2653 (Reactome)
factor IX ProteinP00740 (UniProt)
factor IX activation


ProteinP00740 (UniProt)
factor IXa ComplexREACT_3075 (Reactome)
factor V ProteinP12259 (UniProt)
factor V activation


ProteinP12259 (UniProt)
factor VII ProteinP08709 (UniProt)
factor VIII ComplexREACT_4493 (Reactome)
factor VIII:von

Willebrand factor multimer

ComplexREACT_3248 (Reactome)
factor VIIIa ComplexREACT_4190 (Reactome)
factor VIIIa B A3

acidic polypeptide

ProteinP00451 (UniProt)
factor VIIIa:factor IXa ComplexREACT_3217 (Reactome)
factor VIIa ComplexREACT_2419 (Reactome)
factor Va ComplexREACT_2497 (Reactome)
factor Vi ComplexREACT_4020 (Reactome)
factor X ComplexREACT_3749 (Reactome)
factor X activation


ProteinP00742 (UniProt)
factor XI ComplexREACT_4915 (Reactome)
factor XI:GPIb:GPIX:

GPV complex

ComplexREACT_5726 (Reactome)
factor XII ProteinP00748 (UniProt)
factor XIII ComplexREACT_4285 (Reactome)
factor XIII A chain

activation peptide

ProteinP00488 (UniProt)
factor XIII B chain ProteinP05160 (UniProt)
factor XIII cleaved


ComplexREACT_2648 (Reactome)
factor XIIIa ComplexREACT_4387 (Reactome)
factor XIIa ComplexREACT_4236 (Reactome)
factor XIIa:C1Inh ComplexREACT_2283 (Reactome)
factor XIa:GPIb:GPIX:

GPV complex

ComplexREACT_4765 (Reactome)
factor Xa ComplexREACT_3099 (Reactome)
factor Xa ComplexREACT_5349 (Reactome)


ComplexREACT_5594 (Reactome) Fibrin is a hexamer of two fibrinogen alpha chains, two fibrinogen beta chains, and two fibrinogen gamma chains, held together by disulfide bonds. It is formed in vivo by the thrombin-catalyzed removal of amino terminal fibinopeptides from the A alpha and B beta chains of fibrinogen. This fibrin hexamer ("fibrin monomer") is the subunit that multimerizes to form a fibrin clot ("fibrin multimer").


ComplexREACT_4444 (Reactome) The fibrin "monomers" formed by the action of thrombin on fibrinogen associate spontaneously into multimers. This association can follow several distinct pathways and may be able to form several types of higher-order structures. All of these possibilities are represented in Reactome as a fibrin trimer.
fibrin multimer,


UnknownREACT_2721 (Reactome)


ProteinP02671 (UniProt)
fibrinopeptide B ProteinP02675 (UniProt)
kallikrein ComplexREACT_4124 (Reactome)
kallikrein:C1Inh ComplexREACT_5874 (Reactome)


ComplexREACT_4115 (Reactome)

C1q binding protein tetramer

ComplexREACT_4714 (Reactome)
kininogen ProteinP01042 (UniProt)
kininogen:C1q binding

protein tetramer

ComplexREACT_4804 (Reactome)
prekallikrein ProteinP03952 (UniProt)

kininogen:C1q binding protein tetramer

ComplexREACT_3461 (Reactome)

se dimer

ComplexREACT_5699 (Reactome)
protein C ComplexREACT_4912 (Reactome)
protein C heavy chain

activation peptide

ProteinP04070 (UniProt)
protein S ProteinP07225 (UniProt)
prothrombin (factor


ProteinP00734 (UniProt)
sequestered tissue


ProteinP13726 (UniProt)

activation peptide

ProteinP00734 (UniProt)

antithrombin III: heparin

ComplexREACT_2423 (Reactome)

antithrombin III

ComplexREACT_4175 (Reactome)

antithrombin III: heparin

ComplexREACT_2621 (Reactome)
thrombomodulin ProteinP07204 (UniProt)
tissue factor ProteinP13726 (UniProt)
von Willibrand factor


ComplexREACT_5706 (Reactome)

Annotated Interactions

No annotated interactions

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