COAGULATION CASCADE PATHWAY (PW:0000474)

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Description

In response to vascular injury, the hemostatic system triggers platelet aggregation and initiation of the coagulation cascade, primary and secondary hemostasis, respectively, to prevent bleeding. The coagulation cascade, initiated via tissue factor - the extrinsic pathway, or contact - the intrinsic pathway, converges into a common pathway leading to formation of insoluble fibrin clots. Sequential activation of zymogens (inert enzyme precursors) and cofactors through limited proteolysis yields t

Pathway Diagram:

Elsevier Inc. Serpinc1 ---| F9a Proc_act_diss pF13a F10a F5a F8a F9a vitamin K-dependent proteins, coagulation F7a F7 Proz ---- Serpina10 tenase complex extrinsic tenase complex Klkb1 --+> F12a F12a --+> Klkb1 protein C anticoagulant pathway Serpina10 ---| F10a Proc_act_diss ---| F5a Proc_act_diss ---| F8a F10 ---- TF/F7a complex extrinsic tenase complex --+> F10a F2a ---> protease mediated signaling pathway F10a ---> protease mediated signaling pathway prothrombinase complex --+> F2a F12 tenase complex ---> F10 F12a --+> F11a F12a ---> F11 F3 ---- F7a F11a ---> F9 F11a --+> F9a Vwf F8 F9a ---> F8 F7 --+> F7a F8 ---- Vwf F12 --+> F12a prothrombinase complex ---> F2 fibrin polymerization, cross-linking --+> fibrinolysis pathway Serping1 ---| F12a Serping1 ---| F11a TF/F7a complex TF/F7a complex --+> F9a F2a --+> fibrin pF13 --+> pF13a pKlkb1 Klkb1 Serping1 ---| Klkb1 F2a ---> fibrinogen F5 --+> F5a Tfpi ---| extrinsic tenase complex fibrinolysis pathway ---> fibrin pF13a --+> fibrin polymerization, cross-linking F10 F2 F5 tenase complex --+> F10a Serpinc1 ---| F2a Serpinc1 ---| F10a protease mediated signaling pathway F11 F9a --+> F8a F9 prothrombinase complex F2a pF13 fibrin fibrinogen fibrin polymerization, cross-linking vitamin K cycle pathway kallikrein-kinin cascade pathway F3 Proz Serpina10 Serpinc1 Tfpi Serping1 F12a F11a fibrinolysis pathway
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Genes in Pathway:

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coagulation cascade pathway term browser
Symbol Object Name JBrowse Chr Start Stop Reference
G A2m alpha-2-macroglobulin JBrowse link 4 154,309,426 154,359,138 RGD:6907045
G Apoh apolipoprotein H JBrowse link 10 96,640,013 96,653,939 RGD:1578489
G Bdkrb1 bradykinin receptor B1 JBrowse link 6 129,437,423 129,441,553 RGD:6907045
G Bdkrb2 bradykinin receptor B2 JBrowse link 6 129,399,468 129,429,676 RGD:6907045
G C1qa complement C1q A chain JBrowse link 5 155,261,254 155,264,101 RGD:6907045
G C1qb complement C1q B chain JBrowse link 5 155,246,444 155,251,995 RGD:6907045
G C1qc complement C1q C chain JBrowse link 5 155,255,013 155,258,631 RGD:6907045
G C1r complement C1r JBrowse link 4 157,126,060 157,136,825 RGD:6907045
G C1s complement C1s JBrowse link 4 157,143,592 157,155,592 RGD:6907045
G C2 complement C2 JBrowse link 20 4,542,340 4,561,152 RGD:6907045
G C3 complement C3 JBrowse link 9 9,721,137 9,747,084 RGD:6907045
G C3ar1 complement C3a receptor 1 JBrowse link 4 155,681,767 155,691,240 RGD:6907045
G C4b complement C4B (Chido blood group) JBrowse link 20 4,302,344 4,316,752 RGD:6907045
G C4bpa complement component 4 binding protein, alpha JBrowse link 13 47,342,090 47,377,580 RGD:6907045
G C4bpb complement component 4 binding protein, beta JBrowse link 13 47,387,173 47,398,115 RGD:6907045
G C5 complement C5 JBrowse link 3
3
14,049,993
14,206,466
14,113,931
14,229,141
RGD:6907045
G C5ar1 complement C5a receptor 1 JBrowse link 1 78,186,777 78,195,132 RGD:6907045
G C6 complement C6 JBrowse link 2 54,460,333 54,533,801 RGD:6907045
G C7 complement C7 JBrowse link 2 54,707,621 54,781,624 RGD:6907045
G C8a complement C8 alpha chain JBrowse link 5 124,348,536 124,403,117 RGD:6907045
G C8b complement C8 beta chain JBrowse link 5 124,298,269 124,338,055 RGD:6907045
G C8g complement C8 gamma chain JBrowse link 3 2,724,616 2,727,616 RGD:6907045
G C9 complement C9 JBrowse link 2 55,775,562 55,823,807 RGD:6907045
G Cd46 CD46 molecule JBrowse link 13 113,786,525 113,818,741 RGD:6907045
G Cd55 CD55 molecule, decay accelerating factor for complement JBrowse link 13 47,125,156 47,153,557 RGD:6907045
G Cd59 CD59 molecule JBrowse link 3 94,010,481 94,028,660 RGD:6907045
G Cfb complement factor B JBrowse link 20 4,536,206 4,542,073 RGD:6907045
G Cfd complement factor D JBrowse link 7 12,634,216 12,635,939 RGD:6907045
G Cfh complement factor H JBrowse link 13 56,979,155 57,080,540 RGD:6907045
G Cfi complement factor I JBrowse link 2 235,264,149 235,305,779 RGD:6907045
G Cpb2 carboxypeptidase B2 JBrowse link 15 57,290,849 57,339,762 RGD:6907045
G Cr1l complement C3b/C4b receptor 1 like JBrowse link 13 113,824,886 113,872,097 RGD:6907045
G Cr2 complement C3d receptor 2 JBrowse link 13 113,890,274 113,927,824 RGD:6907045
G F10 coagulation factor X JBrowse link 16 81,803,169 81,822,476 RGD:1580376
RGD:11352277
RGD:11342779
RGD:6907045
RGD:2290183
G F11 coagulation factor XI JBrowse link 16 50,179,458 50,201,644 RGD:2290183
RGD:11352277
RGD:11342779
G F12 coagulation factor XII JBrowse link 17 9,736,577 9,744,420 RGD:2290183
RGD:11352277
RGD:11342779
RGD:6907045
G F13a1 coagulation factor XIII A1 chain JBrowse link 17 28,504,650 28,680,015 RGD:6907045
RGD:11352259
G F13b coagulation factor XIII B chain JBrowse link 13 56,598,891 56,623,132 RGD:6907045
RGD:11352259
G F2 coagulation factor II JBrowse link 3 80,529,468 80,542,993 RGD:1578509
RGD:11352277
RGD:11342779
RGD:6907045
RGD:2290183
G F2r coagulation factor II (thrombin) receptor JBrowse link 2 26,118,760 26,135,340 RGD:6907045
G F3 coagulation factor III, tissue factor JBrowse link 2 225,310,686 225,322,281 RGD:6907045
RGD:11352277
RGD:11342779
G F5 coagulation factor V JBrowse link 13 82,479,997 82,535,540 RGD:1580372
RGD:11352277
RGD:11342779
RGD:2290183
RGD:6907045
G F7 coagulation factor VII JBrowse link 16 81,824,610 81,834,923 RGD:1580376
RGD:11352277
RGD:11342779
RGD:6907045
RGD:2290183
G F8 coagulation factor VIII JBrowse link 18 367,862 399,242 RGD:1582368
RGD:11352277
RGD:11342779
RGD:6907045
RGD:2290183
G F9 coagulation factor IX JBrowse link X 143,097,507 143,141,791 RGD:1580376
RGD:11352277
RGD:11342779
RGD:6907045
RGD:2290183
G Fga fibrinogen alpha chain JBrowse link 2 181,997,562 182,013,726 RGD:6907045
RGD:11352259
G Fgb fibrinogen beta chain JBrowse link 2 182,028,044 182,035,026 RGD:6907045
RGD:11352259
G Fgg fibrinogen gamma chain JBrowse link 2 181,987,080 181,994,523 RGD:6907045
RGD:11352259
G Klkb1 kallikrein B1 JBrowse link 16 50,151,127 50,175,407 RGD:6907045
G Kng2 kininogen 2 JBrowse link 11 81,509,185 81,516,759 RGD:6907045
G Kng2l1 kininogen 2-like 1 RGD:6907045
G Masp1 mannan-binding lectin serine peptidase 1 JBrowse link 11 80,736,424 80,806,278 RGD:6907045
G Masp2 mannan-binding lectin serine peptidase 2 JBrowse link 5 165,415,105 165,429,857 RGD:6907045
G Mbl1 mannose-binding lectin (protein A) 1 JBrowse link 16 18,736,154 18,742,193 RGD:6907045
G Mbl2 mannose binding lectin 2 JBrowse link 1 248,435,069 248,442,669 RGD:6907045
G Plat plasminogen activator, tissue type JBrowse link 16 74,098,263 74,122,897 RGD:6907045
G Plau plasminogen activator, urokinase JBrowse link 15 3,644,296 3,650,765 RGD:6907045
G Plaur plasminogen activator, urokinase receptor JBrowse link 1 81,328,171 81,344,954 RGD:6907045
G Plg plasminogen JBrowse link 1 48,521,828 48,563,895 RGD:6907045
G Proc protein C, inactivator of coagulation factors Va and VIIIa JBrowse link 18 24,918,402 24,928,822 RGD:6907045
RGD:11352277
G Pros1 protein S JBrowse link 7 1,206,648 1,288,140 RGD:6907045
G Proz protein Z, vitamin K-dependent plasma glycoprotein JBrowse link 16 81,784,348 81,797,889 RGD:11352284
G Serpina1 serpin family A member 1 JBrowse link 6 127,610,241 127,632,265 RGD:6907045
G Serpina10 serpin family A member 10 JBrowse link 6 127,500,014 127,508,470 RGD:11352284
G Serpina5 serpin family A member 5 JBrowse link 6 127,753,152 127,772,403 RGD:6907045
G Serpinc1 serpin family C member 1 JBrowse link 13 78,806,107 78,820,375 RGD:6907045
RGD:11352277
G Serpind1 serpin family D member 1 JBrowse link 11 87,913,814 87,924,880 RGD:6907045
G Serpine1 serpin family E member 1 JBrowse link 12 22,641,104 22,651,482 RGD:6907045
G Serpinf2 serpin family F member 2 JBrowse link 10 62,264,247 62,272,353 RGD:6907045
G Serping1 serpin family G member 1 JBrowse link 3 72,161,230 72,171,109 RGD:6907045
RGD:11352277
RGD:11342779
G Tfpi tissue factor pathway inhibitor JBrowse link 3 71,852,738 71,902,127 RGD:6907045
RGD:11352277
RGD:11342779
G Thbd thrombomodulin JBrowse link 3 142,748,673 142,752,325 RGD:6907045
G Vwf von Willebrand factor JBrowse link 4 158,085,059 158,219,525 RGD:6907045

Pathway Gene Annotations

Pathway Annotations Associated with Genes in the coagulation cascade pathway
Pathway TermsGene Symbols
acenocoumarol pharmacodynamics pathwayF10 , F11 , F12 , F13a1 , F13b , F2 , F3 , F5 , F7 , F8 , F9 , Fga , Fgb , Fgg , Klkb1 , Plat , Plg
alteplase pharmacodynamics pathwayF10 , F11 , F12 , F13a1 , F13b , F2 , F3 , F5 , F7 , F8 , F9 , Fga , Fgb , Fgg , Klkb1 , Plat , Plg
Alzheimer disease pathwayA2m
aminocaproic acid pharmacodynamics pathwayF10 , F11 , F12 , F13a1 , F13b , F2 , F3 , F5 , F7 , F8 , F9 , Fga , Fgb , Fgg , Klkb1 , Plat , Plg
anistreplase pharmacodynamics pathwayF10 , F11 , F12 , F13a1 , F13b , F2 , F3 , F5 , F7 , F8 , F9 , Fga , Fgb , Fgg , Klkb1 , Plat , Plg
aprotinin pharmacodynamics pathwayF10 , F11 , F12 , F13a1 , F13b , F2 , F3 , F5 , F7 , F8 , F9 , Fga , Fgb , Fgg , Klkb1 , Plat , Plg
argatroban pharmacodynamics pathwayF10 , F11 , F12 , F13a1 , F13b , F2 , F3 , F5 , F7 , F8 , F9 , Fga , Fgb , Fgg , Klkb1 , Plat , Plg
B cell receptor signaling pathwayCr2
bivalirudin pharmacodynamics pathwayF10 , F11 , F12 , F13a1 , F13b , F2 , F3 , F5 , F7 , F8 , F9 , Fga , Fgb , Fgg , Klkb1 , Plat , Plg
calcium/calcium-mediated signaling pathwayBdkrb1 , Bdkrb2 , F2r
cell-extracellular matrix signaling pathwayVwf
classical complement pathwayC3
coagulation cascade pathwayA2m , Apoh , Bdkrb1 , Bdkrb2 , C1qa , C1qb , C1qc , C1r , C1s , C2 , C3 , C3ar1 , C4b , C4bpa , C4bpb , C5 , C5ar1 , C6 , C7 , C8a , C8b , C8g , C9 , Cd46 , Cd55 , Cd59 , Cfb , Cfd , Cfh , Cfi , Cpb2 , Cr1l , Cr2 , F10 , F11 , F12 , F13a1 , F13b , F2 , F2r , F3 , F5 , F7 , F8 , F9 , Fga , Fgb , Fgg , Klkb1 , Kng2 , Kng2l1 , Masp1 , Masp2 , Mbl1 , Mbl2 , Plat , Plau , Plaur , Plg , Proc , Pros1 , Proz , Serpina1 , Serpina10 , Serpina5 , Serpinc1 , Serpind1 , Serpine1 , Serpinf2 , Serping1 , Tfpi , Thbd , Vwf
complement system pathwayA2m , Bdkrb1 , Bdkrb2 , C1qa , C1qb , C1qc , C1r , C1s , C2 , C3 , C3ar1 , C4b , C4bpa , C4bpb , C5 , C5ar1 , C6 , C7 , C8a , C8b , C8g , C9 , Cd46 , Cd55 , Cd59 , Cfb , Cfd , Cfh , Cfi , Cpb2 , Cr1l , Cr2 , F10 , F12 , F13a1 , F13b , F2 , F2r , F3 , F5 , F7 , F8 , F9 , Fga , Fgb , Fgg , Klkb1 , Kng2 , Kng2l1 , Masp1 , Masp2 , Mbl1 , Mbl2 , Plat , Plau , Plaur , Plg , Proc , Pros1 , Serpina1 , Serpina5 , Serpinc1 , Serpind1 , Serpine1 , Serpinf2 , Serping1 , Tfpi , Thbd , Vwf
dicoumarol pharmacodynamics pathwayF10 , F11 , F12 , F13a1 , F13b , F2 , F3 , F5 , F7 , F8 , F9 , Fga , Fgb , Fgg , Klkb1 , Plat , Plg
endocytosis pathwayF2r
enoxaparin pharmacodynamics pathwayF10 , F11 , F12 , F13a1 , F13b , F2 , F3 , F5 , F7 , F8 , F9 , Fga , Fgb , Fgg , Klkb1 , Plat , Plg , Serpinc1
Entamoeba histolyca infection pathwayC8a , C8b , C8g , C9
ephrin - ephrin receptor bidirectional signaling axisFga , Fgb , Fgg
fibrinolysis pathwayCpb2 , Fga , Fgb , Fgg , Plat , Plau , Plg , Serpina5 , Serpine1 , Serpinf2
fibroblast growth factor signaling pathwayPlau , Plaur
fondaparinux pharmacodynamics pathwayF10 , F11 , F12 , F13a1 , F13b , F2 , F3 , F5 , F7 , F8 , F9 , Fga , Fgb , Fgg , Klkb1 , Plat , Plg , Serpinc1
forkhead class A signaling pathwayC4bpb , F2 , Serpina1
G protein mediated signaling pathwayF2r
G protein mediated signaling pathway via Galphai familyBdkrb1 , Bdkrb2
G protein mediated signaling pathway via Galphaq familyBdkrb1 , Bdkrb2
glypican signaling pathwaySerpinc1
heparin pharmacodynamics pathwayF10 , F11 , F12 , F13a1 , F13b , F2 , F3 , F5 , F7 , F8 , F9 , Fga , Fgb , Fgg , Klkb1 , Plat , Plg , Serpinc1
hypoxia inducible factor pathwaySerpine1
immune response pathwayA2m
inflammatory response pathwayA2m
influenza A virus infection pathwayPlg
interleukin-6 signaling pathwayA2m , Fgg
kallikrein-kinin cascade pathwayA2m , Bdkrb1 , Bdkrb2 , F12 , Klkb1 , Kng2 , Serping1
kinin signaling pathwayBdkrb1 , Bdkrb2 , Kng2
lectin complement pathwayMbl2
Leishmania infection pathwayC3
measles infection pathwayCd46
Mycobacterium tuberculosis infection pathwayC3
myocarditis pathwayCd55
oxygen homeostasis pathwaySerpine1
p53 signaling pathwaySerpine1
phagocytosis pathwayC1r , C3 , Mbl1 , Mbl2
phenindione pharmacodynamics pathwayF10 , F11 , F12 , F13a1 , F13b , F2 , F3 , F5 , F7 , F8 , F9 , Fga , Fgb , Fgg , Klkb1 , Plat , Plg
platelet aggregation pathwayVwf
prion diseases pathwayC1qa , C1qb , C1qc , C5 , C6 , C7 , C8a , C8b , C8g , C9
protease mediated signaling via protease-activated receptor 1F2 , F2r , Proc
protease mediated signaling via protease-activated receptor 2F10
protease mediated signaling via protease-activated receptor 3F2
protease mediated signaling via protease-activated receptor 4F2
protein C anticoagulant pathwayF2 , F5 , Proc , Pros1 , Thbd
reteplase pharmacodynamics pathwayF10 , F11 , F12 , F13a1 , F13b , F2 , F3 , F5 , F7 , F8 , F9 , Fga , Fgb , Fgg , Klkb1 , Plat , Plg
Staphylococcus aureus infection pathwayC1qa , C1qb , C1qc , C1r , C1s , C2 , C3 , C3ar1 , C4b , C5 , C5ar1 , Cfb , Cfd , Cfh , Cfi , Fgg , Masp1 , Masp2 , Mbl1 , Mbl2 , Plg
syndecan signaling pathwayF2 , Kng2 , Plg , Tfpi
systemic lupus erythematosus pathwayC1qa , C1qb , C1qc , C1r , C1s , C2 , C3 , C4b , C5 , C6 , C7 , C8a , C8b , C8g , C9
tranexamic acid pharmacodynamics pathwayF10 , F11 , F12 , F13a1 , F13b , F2 , F3 , F5 , F7 , F8 , F9 , Fga , Fgb , Fgg , Klkb1 , Plat , Plg
transforming growth factor-beta Smad dependent signaling pathwayA2m
Trypanosoma cruzi infection pathwayBdkrb2 , C1qa , C1qb , C1qc , C3 , Serpine1
vitamin K metabolic pathwayF2
warfarin pharmacodynamics pathwayF10 , F11 , F12 , F13a1 , F13b , F2 , F3 , F5 , F7 , F8 , F9 , Fga , Fgb , Fgg , Klkb1 , Plat , Plg
Phenotype Annotations Associated with Genes in the coagulation cascade pathway

References Associated with the coagulation cascade pathway:

Ontology Path Diagram:

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