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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  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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