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

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1, 258, 62-6440-448636-3934, 47-5323-2517, 2076-7835, 38, 45, 4682172254-5776, 80, 818258-617577, 7967, 68, 71, 73856, 82667-7286231324, 28, 292728, 308665, 6631867417261332-358483cytoplasmkininogenthrombomodulinfactor XIII cleaved tetramerfactor VIII:von Willebrand factor multimerfactor XIIIafactor XIa:GPIb:GPIX:GPV complexfibrin monomerfactor VIIIa:factor IXaPlatelet Factor 4factor IXfactor XIIprolylcarboxypeptidase dimeralpha2-macroglobulinfactor VIIIakallikrein:kininogen:C1q binding protein tetramersequestered tissue factorfactor Vafactor XIII A chain activation peptidekallikrein:C1InhkallikreinZn++factor XIIafactor Xaactivated protein Cfibrinopeptide Aprothrombin (factor II)kininogen:C1q binding protein tetramerfactor XI:GPIb:GPIX:GPV complexfibrinopeptide Bthrombin activation peptidethrombin:cleaved antithrombin III:heparinfactor VIIfactor VIIIantithrombin III:heparinfactor VIIIa B A3 acidic polypeptideTFPIfactor IXaFibrinogenfibrin multimer, crosslinkedactivated protein Cvon Willibrand factor multimerfactor XIIIGPIb:GPIX:GPV complexprotein C heavy chain activation peptideTF:F7tissue factorVa:Xa complex (prothrombinase)Activated thrombin (factor IIa)CalciumBradykininprekallikreinkallikrein:alpha2-macroglobulinantithrombin IIIC1InhTFPI:TF:F7a:factor Xaactivated kininogen:C1q binding protein tetramerfactor VIIaprotein Cprekallikrein:kininogen:C1q binding protein tetramerthrombin:cleaved antithrombin IIINH4+factor V activation peptidefactor ViTF:F7afactor X activation peptidefactor XaC1q binding protein tetramerCalciumCalciumfactor XIIa:C1Inhactivated thrombin:thrombomodulinfactor Xfactor IX activation peptidethrombin:antithrombin III:heparinfibrin multimerprotein SkininogenCalciumfactor Vfactor XIII B chainfactor XIHeparin17618, 1910-128, 93, 418, 1915718-202113138510, 121618-205, 65, 68143, 4


Description

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. Kisiel W.; ''Human plasma protein C: isolation, characterization, and mechanism of activation by alpha-thrombin.''; PubMed Europe PMC Scholia
  2. 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
  3. de Maat S, Björkqvist J, Suffritti C, Wiesenekker CP, Nagtegaal W, Koekman A, van Dooremalen S, Pasterkamp G, de Groot PG, Cicardi M, Renné T, Maas C.; ''Plasmin is a natural trigger for bradykinin production in patients with hereditary angioedema with factor XII mutations.''; PubMed Europe PMC Scholia
  4. Mann KG, Butenas S, Brummel K.; ''The dynamics of thrombin formation.''; PubMed Europe PMC Scholia
  5. 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
  6. Kothari H, Pendurthi UR, Rao LV.; ''Analysis of tissue factor expression in various cell model systems: cryptic vs. active.''; PubMed Europe PMC Scholia
  7. Broze GJ.; ''Binding of human factor VII and VIIa to monocytes.''; PubMed Europe PMC Scholia
  8. 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
  9. 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
  10. 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
  11. Bock SC, Wion KL, Vehar GA, Lawn RM.; ''Cloning and expression of the cDNA for human antithrombin III.''; PubMed Europe PMC Scholia
  12. 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
  13. Foster D, Davie EW.; ''Characterization of a cDNA coding for human protein C.''; PubMed Europe PMC Scholia
  14. 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
  15. 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
  16. 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
  17. 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
  18. Suzuki K, Nishioka J, Kusumoto H, Hashimoto S.; ''Mechanism of inhibition of activated protein C by protein C inhibitor.''; PubMed Europe PMC Scholia
  19. 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
  20. 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
  21. Ruf W, Kalnik MW, Lund-Hansen T, Edgington TS.; ''Characterization of factor VII association with tissue factor in solution. High and low affinity calcium binding sites in factor VII contribute to functionally distinct interactions.''; PubMed Europe PMC Scholia
  22. Orfeo T, Brufatto N, Nesheim ME, Xu H, Butenas S, Mann KG.; ''The factor V activation paradox.''; PubMed Europe PMC Scholia
  23. 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
  24. 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
  25. 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
  26. 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
  27. 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
  28. Rao LV, Pendurthi UR.; ''Regulation of tissue factor coagulant activity on cell surfaces.''; PubMed Europe PMC Scholia
  29. 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
  30. Pipe SW, Saenko EL, Eickhorst AN, Kemball-Cook G, Kaufman RJ.; ''Hemophilia A mutations associated with 1-stage/2-stage activity discrepancy disrupt protein-protein interactions within the triplicated A domains of thrombin-activated factor VIIIa.''; PubMed Europe PMC Scholia
  31. 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
  32. 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
  33. Ivanov I, Matafonov A, Sun MF, Mohammed BM, Cheng Q, Dickeson SK, Kundu S, Verhamme IM, Gruber A, McCrae K, Gailani D.; ''A mechanism for hereditary angioedema with normal C1 inhibitor: an inhibitory regulatory role for the factor XII heavy chain.''; PubMed Europe PMC Scholia
  34. Kaufman RJ, Pipe SW, Tagliavacca L, Swaroop M, Moussalli M.; ''Biosynthesis, assembly and secretion of coagulation factor VIII.''; PubMed Europe PMC Scholia
  35. 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
  36. Pixley RA, Schapira M, Colman RW.; ''The regulation of human factor XIIa by plasma proteinase inhibitors.''; PubMed Europe PMC Scholia
  37. DiScipio RG, Davie EW.; ''Characterization of protein S, a gamma-carboxyglutamic acid containing protein from bovine and human plasma.''; PubMed Europe PMC Scholia
  38. Bouma BN, Griffin JH.; ''Human blood coagulation factor XI. Purification, properties, and mechanism of activation by activated factor XII.''; PubMed Europe PMC Scholia
  39. Esmon CT.; ''The roles of protein C and thrombomodulin in the regulation of blood coagulation.''; PubMed Europe PMC Scholia
  40. Butenas S, Mann KG.; ''Kinetics of human factor VII activation.''; PubMed Europe PMC Scholia
  41. Lawson JH, Mann KG.; ''Cooperative activation of human factor IX by the human extrinsic pathway of blood coagulation.''; PubMed Europe PMC Scholia
  42. Degen SJ, Davie EW.; ''Nucleotide sequence of the gene for human prothrombin.''; PubMed Europe PMC Scholia
  43. 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
  44. 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
  45. 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
  46. 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
  47. 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
  48. Simioni P, Tormene D, Tognin G, Gavasso S, Bulato C, Iacobelli NP, Finn JD, Spiezia L, Radu C, Arruda VR.; ''X-linked thrombophilia with a mutant factor IX (factor IX Padua).''; PubMed Europe PMC Scholia
  49. 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
  50. Grover SP, Mackman N.; ''Tissue Factor: An Essential Mediator of Hemostasis and Trigger of Thrombosis.''; PubMed Europe PMC Scholia
  51. Shariat-Madar Z, Mahdi F, Schmaier AH.; ''Identification and characterization of prolylcarboxypeptidase as an endothelial cell prekallikrein activator.''; PubMed Europe PMC Scholia
  52. 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
  53. Mann KG, Kalafatis M.; ''Factor V: a combination of Dr Jekyll and Mr Hyde.''; PubMed Europe PMC Scholia
  54. Mohammed BM, Matafonov A, Ivanov I, Sun MF, Cheng Q, Dickeson SK, Li C, Sun D, Verhamme IM, Emsley J, Gailani D.; ''An update on factor XI structure and function.''; PubMed Europe PMC Scholia
  55. Kurosawa S, Stearns-Kurosawa DJ, Hidari N, Esmon CT.; ''Identification of functional endothelial protein C receptor in human plasma.''; PubMed Europe PMC Scholia
  56. Ichinose A, Hendrickson LE, Fujikawa K, Davie EW.; ''Amino acid sequence of the a subunit of human factor XIII.''; PubMed Europe PMC Scholia
  57. 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
  58. Rawala-Sheikh R, Ahmad SS, Ashby B, Walsh PN.; ''Kinetics of coagulation factor X activation by platelet-bound factor IXa.''; PubMed Europe PMC Scholia
  59. 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
  60. Lollar P, Hill-Eubanks DC, Parker CG.; ''Association of the factor VIII light chain with von Willebrand factor.''; PubMed Europe PMC Scholia
  61. 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
  62. Mann KG, Jenny RJ, Krishnaswamy S.; ''Cofactor proteins in the assembly and expression of blood clotting enzyme complexes.''; PubMed Europe PMC Scholia
  63. 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
  64. 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
  65. Kaufman RJ, Wasley LC, Dorner AJ.; ''Synthesis, processing, and secretion of recombinant human factor VIII expressed in mammalian cells.''; PubMed Europe PMC Scholia
  66. 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
  67. Turner NA, Moake JL.; ''Factor VIII Is Synthesized in Human Endothelial Cells, Packaged in Weibel-Palade Bodies and Secreted Bound to ULVWF Strings.''; PubMed Europe PMC Scholia
  68. Vadivel K, Bajaj SP.; ''Structural biology of factor VIIa/tissue factor initiated coagulation.''; PubMed Europe PMC Scholia
  69. Fujikawa K, McMullen BA.; ''Amino acid sequence of human beta-factor XIIa.''; PubMed Europe PMC Scholia
  70. 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
  71. Odya CE, Marinkovic DV, Hammon KJ, Stewart TA, Erdös EG.; ''Purification and properties of prolylcarboxypeptidase (angiotensinase C) from human kidney.''; PubMed Europe PMC Scholia
  72. 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
  73. 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
  74. 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
  75. Walz DA, Hewett-Emmett D, Seegers WH.; ''Amino acid sequence of human prothrombin fragments 1 and 2.''; PubMed Europe PMC Scholia
  76. Geng Y, Verhamme IM, Messer A, Sun MF, Smith SB, Bajaj SP, Gailani D.; ''A sequential mechanism for exosite-mediated factor IX activation by factor XIa.''; PubMed Europe PMC Scholia
  77. 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
  78. McMullen BA, Fujikawa K.; ''Amino acid sequence of the heavy chain of human alpha-factor XIIa (activated Hageman factor).''; PubMed Europe PMC Scholia
  79. Fay PJ, Haidaris PJ, Smudzin TM.; ''Human factor VIIIa subunit structure. Reconstruction of factor VIIIa from the isolated A1/A3-C1-C2 dimer and A2 subunit.''; PubMed Europe PMC Scholia
  80. 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
  81. Hill-Eubanks DC, Parker CG, Lollar P.; ''Differential proteolytic activation of factor VIII-von Willebrand factor complex by thrombin.''; PubMed Europe PMC Scholia
  82. 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
  83. 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
  84. Kaufman RJ, Wasley LC, Furie BC, Furie B, Shoemaker CB.; ''Expression, purification, and characterization of recombinant gamma-carboxylated factor IX synthesized in Chinese hamster ovary cells.''; PubMed Europe PMC Scholia
  85. 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
  86. 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
  87. Schreiber AD, Kaplan AP, Austen KF.; ''Inhibition by C1INH of Hagemann factor fragment activation of coagulation, fibrinolysis, and kinin generation.''; PubMed Europe PMC Scholia
  88. Celie PH, Van Stempvoort G, Jorieux S, Mazurier C, Van Mourik JA, Mertens K.; ''Substitution of Arg527 and Arg531 in factor VIII associated with mild haemophilia A: characterization in terms of subunit interaction and cofactor function.''; PubMed Europe PMC Scholia
  89. 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
  90. Bhanwra S, Ahluwalia K.; ''The new factor Xa inhibitor: Apixaban.''; PubMed Europe PMC Scholia
  91. Sorensen AB, Madsen JJ, Frimurer TM, Overgaard MT, Gandhi PS, Persson E, Olsen OH.; ''Allostery in Coagulation Factor VIIa Revealed by Ensemble Refinement of Crystallographic Structures.''; PubMed Europe PMC Scholia
  92. Prandini MH, Reboul A, Colomb MG.; ''Biosynthesis of complement C1 inhibitor by Hep G2 cells. Reactivity of different glycosylated forms of the inhibitor with C1s.''; PubMed Europe PMC Scholia
  93. Leytus SP, Chung DW, Kisiel W, Kurachi K, Davie EW.; ''Characterization of a cDNA coding for human factor X.''; PubMed Europe PMC Scholia
  94. de Maat S, Clark CC, Boertien M, Parr N, Sanrattana W, Hofman ZLM, Maas C.; ''Factor XII truncation accelerates activation in solution.''; PubMed Europe PMC Scholia
  95. Griffin JH, Fernández JA, Gale AJ, Mosnier LO.; ''Activated protein C.''; PubMed Europe PMC Scholia
  96. Gladwell TD.; ''Bivalirudin: a direct thrombin inhibitor.''; PubMed Europe PMC Scholia
  97. McMullen BA, Fujikawa K, Kisiel W.; ''The occurrence of beta-hydroxyaspartic acid in the vitamin K-dependent blood coagulation zymogens.''; PubMed Europe PMC Scholia
  98. Kurachi K, Davie EW.; ''Activation of human factor XI (plasma thromboplastin antecedent) by factor XIIa (activated Hageman factor).''; PubMed Europe PMC Scholia
  99. 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
  100. Minor C, Tellor KB, Armbruster AL.; ''Edoxaban, a Novel Oral Factor Xa Inhibitor.''; PubMed Europe PMC Scholia
  101. Lenting PJ, van Mourik JA, Mertens K.; ''The life cycle of coagulation factor VIII in view of its structure and function.''; PubMed Europe PMC Scholia
  102. 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
  103. Mushunje A, Zhou A, Carrell RW, Huntington JA.; ''Heparin-induced substrate behavior of antithrombin Cambridge II.''; PubMed Europe PMC Scholia
  104. 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
  105. 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
  106. Hakeos WH, Miao H, Sirachainan N, Kemball-Cook G, Saenko EL, Kaufman RJ, Pipe SW.; ''Hemophilia A mutations within the factor VIII A2-A3 subunit interface destabilize factor VIIIa and cause one-stage/two-stage activity discrepancy.''; PubMed Europe PMC Scholia
  107. 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
  108. 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
  109. 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
  110. 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
  111. 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
  112. 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
  113. Bondarenko M, Curti C, Montana M, Rathelot P, Vanelle P.; ''Efficacy and toxicity of factor Xa inhibitors.''; PubMed Europe PMC Scholia
  114. 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
  115. 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
  116. Silverberg M, Dunn JT, Garen L, Kaplan AP.; ''Autoactivation of human Hageman factor. Demonstration utilizing a synthetic substrate.''; PubMed Europe PMC Scholia
  117. 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
  118. Fay PJ, Smudzin TM.; ''Characterization of the interaction between the A2 subunit and A1/A3-C1-C2 dimer in human factor VIIIa.''; PubMed Europe PMC Scholia
  119. 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
  120. Griffin JH, Cochrane CG.; ''Mechanisms for the involvement of high molecular weight kininogen in surface-dependent reactions of Hageman factor.''; PubMed Europe PMC Scholia
  121. 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
  122. Broze GJ, Girard TJ, Novotny WF.; ''Regulation of coagulation by a multivalent Kunitz-type inhibitor.''; PubMed Europe PMC Scholia
  123. 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
  124. Davie EW, Fujikawa K, Kisiel W.; ''The coagulation cascade: initiation, maintenance, and regulation.''; PubMed Europe PMC Scholia
  125. Rapaport SI, Rao LV.; ''The tissue factor pathway: how it has become a "prima ballerina".''; PubMed Europe PMC Scholia
  126. Church FC, Noyes CM, Griffith MJ.; ''Inhibition of chymotrypsin by heparin cofactor II.''; PubMed Europe PMC Scholia
  127. Lewis SD, Janus TJ, Lorand L, Shafer JA.; ''Regulation of formation of factor XIIIa by its fibrin substrates.''; PubMed Europe PMC Scholia
  128. 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
  129. Pan S, Iannotti MJ, Sifers RN.; ''Analysis of serpin secretion, misfolding, and surveillance in the endoplasmic reticulum.''; PubMed Europe PMC Scholia
  130. Nemerson Y.; ''Tissue factor and hemostasis.''; PubMed Europe PMC Scholia
  131. 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
  132. Pipe SW, Eickhorst AN, McKinley SH, Saenko EL, Kaufman RJ.; ''Mild hemophilia A caused by increased rate of factor VIII A2 subunit dissociation: evidence for nonproteolytic inactivation of factor VIIIa in vivo.''; PubMed Europe PMC Scholia
  133. 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
  134. 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
  135. Hoeben RC, Fallaux FJ, Cramer SJ, van den Wollenberg DJ, van Ormondt H, Briët E, van der Eb AJ.; ''Expression of the blood-clotting factor-VIII cDNA is repressed by a transcriptional silencer located in its coding region.''; PubMed Europe PMC Scholia
  136. Gilbert GE, Furie BC, Furie B.; ''Binding of human factor VIII to phospholipid vesicles.''; PubMed Europe PMC Scholia
  137. Schmaier AH.; ''The physiologic basis of assembly and activation of the plasma kallikrein/kinin system.''; PubMed Europe PMC Scholia
  138. 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
  139. 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
  140. Butkowski RJ, Elion J, Downing MR, Mann KG.; ''Primary structure of human prethrombin 2 and alpha-thrombin.''; PubMed Europe PMC Scholia
  141. Shariat-Madar Z, Mahdi F, Schmaier AH.; ''Recombinant prolylcarboxypeptidase activates plasma prekallikrein.''; PubMed Europe PMC Scholia
  142. Greengard JS, Heeb MJ, Ersdal E, Walsh PN, Griffin JH.; ''Binding of coagulation factor XI to washed human platelets.''; PubMed Europe PMC Scholia
  143. Holmer E, Söderberg K, Bergqvist D, Lindahl U.; ''Heparin and its low molecular weight derivatives: anticoagulant and antithrombotic properties.''; PubMed Europe PMC Scholia
  144. 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
  145. Kurachi K, Kurachi S, Furukawa M, Yao SN.; ''Biology of factor IX.''; PubMed Europe PMC Scholia

History

View all...
CompareRevisionActionTimeUserComment
123618view08:20, 7 August 2022EgonwModified title
114744view16:23, 25 January 2021ReactomeTeamReactome version 75
113188view11:25, 2 November 2020ReactomeTeamReactome version 74
112416view15:35, 9 October 2020ReactomeTeamReactome version 73
101320view11:21, 1 November 2018ReactomeTeamreactome version 66
100857view20:53, 31 October 2018ReactomeTeamreactome version 65
100398view19:27, 31 October 2018ReactomeTeamreactome version 64
99946view16:11, 31 October 2018ReactomeTeamreactome version 63
99502view14:44, 31 October 2018ReactomeTeamreactome version 62 (2nd attempt)
94025view13:52, 16 August 2017ReactomeTeamreactome version 61
93645view11:29, 9 August 2017ReactomeTeamreactome version 61
87449view13:55, 22 July 2016MkutmonOntology Term : 'coagulation cascade pathway' added !
86761view09:25, 11 July 2016ReactomeTeamreactome version 56
83143view10:09, 18 November 2015ReactomeTeamVersion54
81490view13:01, 21 August 2015ReactomeTeamVersion53
76965view08:24, 17 July 2014ReactomeTeamFixed remaining interactions
76670view12:03, 16 July 2014ReactomeTeamFixed remaining interactions
75999view10:05, 11 June 2014ReactomeTeamRe-fixing comment source
75702view11:04, 10 June 2014ReactomeTeamReactome 48 Update
75538view19:27, 9 June 2014MaintBotchanged description source
75512view12:25, 5 June 2014AnweshaUpdated in Reactome48
75058view13:56, 8 May 2014AnweshaFixing comment source for displaying WikiPathways description
74702view08:46, 30 April 2014ReactomeTeamReactome46
73650view00:25, 12 February 2014AriuttaRemoved GroupRef="group_comp_1316" because there is no Group with GroupId="group_comp_1316"
73634view20:28, 10 February 2014KhanspersReverted to version '20:24, 8 February 2014' by Khanspers
73633view20:24, 10 February 2014Khanspersremoved cell shape
73632view20:22, 10 February 2014Khanspersremoved all groups to possibly resolve crash
73624view20:24, 8 February 2014MaintBotTrying out new gpml conversion to resolve crash of new pvjs viewer
69011view17:46, 8 July 2013MaintBotUpdated to 2013 gpml schema
42041view21:52, 4 March 2011MaintBotAutomatic update
39844view05:52, 21 January 2011MaintBotNew pathway

External references

DataNodes

View all...
NameTypeDatabase referenceComment
Activated thrombin

(factor IIa)

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

tetramer

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.
GPIb:GPIX:GPV

complex

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

(prothrombinase)

ComplexREACT_5441 (Reactome)
Zn++ Metabolite29105 (ChEBI)
activated

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)
alpha2-

macroglobulin

ComplexREACT_3449 (Reactome)
antithrombin

III

ProteinP01008 (UniProt)
antithrombin III:

heparin

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

peptide

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

peptide

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

peptide

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

tetramer

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)
fibrin

monomer

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").
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,

crosslinked

UnknownREACT_2721 (Reactome)
fibrinopeptide

A

ProteinP02671 (UniProt)
fibrinopeptide B ProteinP02675 (UniProt)
kallikrein ComplexREACT_4124 (Reactome)
kallikrein:C1Inh ComplexREACT_5874 (Reactome)
kallikrein:alpha2-

macroglobulin

ComplexREACT_4115 (Reactome)
kallikrein:kininogen:

C1q binding protein tetramer

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

protein tetramer

ComplexREACT_4804 (Reactome)
prekallikrein ProteinP03952 (UniProt)
prekallikrein:

kininogen:C1q binding protein tetramer

ComplexREACT_3461 (Reactome)
prolylcarboxypeptida

se dimer

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

activation peptide

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

II)

ProteinP00734 (UniProt)
sequestered tissue

factor

ProteinP13726 (UniProt)
thrombin

activation peptide

ProteinP00734 (UniProt)
thrombin:

antithrombin III: heparin

ComplexREACT_2423 (Reactome)
thrombin:cleaved

antithrombin III

ComplexREACT_4175 (Reactome)
thrombin:cleaved

antithrombin III: heparin

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

multimer

ComplexREACT_5706 (Reactome)

Annotated Interactions

No annotated interactions

Retrieved from "https://classic.wikipathways.org/index.php/Pathway:WP1818"
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