KEGG   PATHWAY: tfn04610
Entry
tfn04610                    Pathway                                
Name
Complement and coagulation cascades - Trachypithecus francoisi (Francois's langur)
Description
The complement system is a proteolytic cascade in blood plasma and a mediator of innate immunity, a nonspecific defense mechanism against pathogens. There are three pathways of complement activation: the classical pathway, the lectin pathway, and the alternative pathway. All of these pathways generate a crucial enzymatic activity that, in turn, generates the effector molecules of complement. The main consequences of complement activation are the opsonization of pathogens, the recruitment of inflammatory and immunocompetent cells, and the direct killing of pathogens. Blood coagulation is another series of proenzyme-to-serine protease conversions, culminating the formation of thrombin, the enzyme responsible for the conversion of soluble fibrinogen to the insoluble fibrin clot. Protease-activated receptors, such as those activated by thrombin, are members of G protein-coupled receptors and function as a mediator of innate immunity. The kallikrein-kinin system is an endogenous metabolic cascade, triggering of which results in the release of vasoactive kinins (bradykinin-related peptides). Kinin peptides are implicated in many physiological and pathological processes including the regulation of blood pressure and sodium homeostasis, inflammatory processes, and the cardioprotective effects of preconditioning.
Class
Organismal Systems; Immune system
Pathway map
tfn04610  Complement and coagulation cascades
tfn04610

Organism
Trachypithecus francoisi (Francois's langur) [GN:tfn]
Gene
117063480  F7; coagulation factor VII isoform X1 [KO:K01320] [EC:3.4.21.21]
117076734  F3; tissue factor isoform X1 [KO:K03901]
117065332  F12; coagulation factor XII isoform X1 [KO:K01328] [EC:3.4.21.38]
117086227  F11; coagulation factor XI isoform X1 [KO:K01323] [EC:3.4.21.27]
117078021  F9; coagulation factor IX [KO:K01321] [EC:3.4.21.22]
117090655  VWF; von Willebrand factor [KO:K03900]
117078349  F8; LOW QUALITY PROTEIN: coagulation factor VIII [KO:K03899]
117063476  F10; coagulation factor X [KO:K01314] [EC:3.4.21.6]
117076423  F5; coagulation factor V [KO:K03902]
117081636  F2; prothrombin [KO:K01313] [EC:3.4.21.5]
117086051  FGA; fibrinogen alpha chain [KO:K03903]
117086050  FGB; fibrinogen beta chain [KO:K03904]
117086053  FGG; fibrinogen gamma chain isoform X1 [KO:K03905]
117086671  F13A1; coagulation factor XIII A chain [KO:K03917] [EC:2.3.2.13]
117075780  F13B; coagulation factor XIII B chain [KO:K03906]
117065283  F2R; proteinase-activated receptor 1 isoform X1 [KO:K03914]
117065282  F2RL2; proteinase-activated receptor 3 [KO:K04235]
117074734  F2RL3; proteinase-activated receptor 4 [KO:K04236]
117063285  CPB2; carboxypeptidase B2 isoform X1 [KO:K01300] [EC:3.4.17.20]
117068372  THBD; thrombomodulin [KO:K03907]
117068690  PROCR; endothelial protein C receptor [KO:K06557]
117095160  PROC; vitamin K-dependent protein C isoform X1 [KO:K01344] [EC:3.4.21.69]
117083637  PROS1; vitamin K-dependent protein S [KO:K03908]
117072464  SERPINE1; plasminogen activator inhibitor 1 [KO:K03982]
117062029  SERPINB2; plasminogen activator inhibitor 2 [KO:K19821]
117071471  SERPINA5; plasma serine protease inhibitor [KO:K03913]
117094453  SERPINE2; glia-derived nexin isoform X1 [KO:K16643]
117095617  PLAT; tissue-type plasminogen activator [KO:K01343] [EC:3.4.21.68]
117082114  PLAU; urokinase-type plasminogen activator isoform X1 [KO:K01348] [EC:3.4.21.73]
117091912  PLAUR; urokinase plasminogen activator surface receptor [KO:K03985]
117096916  PLG; plasminogen [KO:K01315] [EC:3.4.21.7]
117086226  KLKB1; plasma kallikrein isoform X1 [KO:K01324] [EC:3.4.21.34]
117084346  KNG1; kininogen-1 [KO:K03898]
117071446  B2 bradykinin receptor isoform X1 [KO:K03916]
117094749  TFPI; tissue factor pathway inhibitor isoform X1 [KO:K03909]
117071481  SERPINA1; alpha-1-antitrypsin [KO:K03984]
117074627  SERPINC1; antithrombin-III [KO:K03911]
117096620  SERPIND1; heparin cofactor 2 [KO:K03912]
117079824  SERPING1; plasma protease C1 inhibitor [KO:K04001]
117067946  SERPINF2; alpha-2-antiplasmin [KO:K03983]
117090784  alpha-2-macroglobulin isoform X1 [KO:K03910]
117093156  C1QA; complement C1q subcomponent subunit A [KO:K03986]
117093154  C1QB; complement C1q subcomponent subunit B [KO:K03987]
117093155  C1QC; complement C1q subcomponent subunit C isoform X1 [KO:K03988]
117090591  complement C1r subcomponent isoform X1 [KO:K01330] [EC:3.4.21.41]
117090593  complement C1s subcomponent [KO:K01331] [EC:3.4.21.42]
117082309  mannose-binding protein A-like [KO:K03991]
117085029  mannose-binding protein A-like [KO:K03991]
117085810  MBL2; mannose-binding protein C [KO:K03991]
117084356  MASP1; mannan-binding lectin serine protease 1 isoform X1 [KO:K03992] [EC:3.4.21.-]
117093329  MASP2; mannan-binding lectin serine protease 2 isoform X1 [KO:K03993] [EC:3.4.21.104]
117086984  complement C4-B [KO:K03989]
117086977  C2; complement C2 isoform X1 [KO:K01332] [EC:3.4.21.43]
117075829  C3; complement C3 [KO:K03990]
117086976  CFB; complement factor B [KO:K01335] [EC:3.4.21.47]
117075456  CFD; complement factor D isoform X1 [KO:K01334] [EC:3.4.21.46]
117098742  C5; complement C5 isoform X1 [KO:K03994]
117065060  C6; LOW QUALITY PROTEIN: complement component C6 [KO:K03995]
117065057  C7; complement component C7 isoform X1 [KO:K03996]
117091974  C8A; complement component C8 alpha chain [KO:K03997]
117091975  C8B; complement component C8 beta chain [KO:K03998]
117099134  C8G; complement component C8 gamma chain [KO:K03999]
117065049  C9; complement component C9 [KO:K04000]
117090531  C3AR1; C3a anaphylatoxin chemotactic receptor [KO:K04009]
117078546  VSIG4; V-set and immunoglobulin domain-containing protein 4 isoform X1 [KO:K19822]
117075393  complement receptor type 1 isoform X1 [KO:K04011]
117075394  complement receptor type 1-like [KO:K04011]
117074329  CR2; complement receptor type 2 isoform X1 [KO:K04012]
117087699  ITGAM; integrin alpha-M isoform X1 [KO:K06461]
117096348  ITGB2; LOW QUALITY PROTEIN: integrin beta-2 [KO:K06464]
117090698  LOW QUALITY PROTEIN: integrin beta-2-like [KO:K06464]
117087704  ITGAX; LOW QUALITY PROTEIN: integrin alpha-X [KO:K06462]
117091470  C5AR1; C5a anaphylatoxin chemotactic receptor 1 [KO:K04010]
117075549  C4BPA; C4b-binding protein alpha chain isoform X1 [KO:K04002]
117075550  LOW QUALITY PROTEIN: zona pellucida sperm-binding protein 3 receptor-like [KO:K04002]
117075280  C4BPB; C4b-binding protein beta chain isoform X1 [KO:K04003]
117085781  CFI; complement factor I isoform X1 [KO:K01333] [EC:3.4.21.45]
117076781  CFH; complement factor H isoform X1 [KO:K04004]
117075396  membrane cofactor protein isoform X1 [KO:K04007]
117075399  membrane cofactor protein-like [KO:K04007]
117075552  CD55; complement decay-accelerating factor isoform X1 [KO:K04006]
117076783  CFHR2; complement factor H-related protein 2 [KO:K23815]
117076782  CFHR5; complement factor H-related protein 5 [KO:K23817]
117081502  CD59; CD59 glycoprotein [KO:K04008]
117093909  CLU; clusterin isoform X1 [KO:K17252]
117067614  VTN; vitronectin [KO:K06251]
Compound
C00290  Fibrin
C00306  Bradykinin
C00471  Plasmin
C00752  Thrombin
C01065  Coagulation factor Xa
C01715  Plasma kallikrein
C02002  C3a anaphylatoxin
C03259  Coagulation factor IXa
C03262  Coagulation factor XIa
C03378  Coagulation factor VIIa
C03379  Coagulation factor XIIa
C03780  Coagulation factor Va
C04001  Coagulation factor VIIIa
C16510  C5a anaphylatoxin
C22531  Complement component 2a
C22532  Complement component 2b
C22533  Complement component 3b
C22534  Complement component 3f
C22535  Complement iC3b
C22536  Complement component 3dg
C22537  Complement component 3c
C22538  Complement component 3d
C22539  Complement C3(H2O)
C22540  Complement factor Bb
C22541  Complement component 4a
C22542  Complement component 4b
C22543  Complement component 4c
C22544  Complement component 4d
C22545  Complement component 5b
C22546  Coagulation factor XIIIa
C22547  APC
Reference
  Authors
Bajic G, Degn SE, Thiel S, Andersen GR
  Title
Complement activation, regulation, and molecular basis for complement-related diseases.
  Journal
EMBO J 34:2735-57 (2015)
DOI:10.15252/embj.201591881
Reference
  Authors
Mathern DR, Heeger PS
  Title
Molecules Great and Small: The Complement System.
  Journal
Clin J Am Soc Nephrol 10:1636-50 (2015)
DOI:10.2215/CJN.06230614
Reference
  Authors
Merle NS, Church SE, Fremeaux-Bacchi V, Roumenina LT
  Title
Complement System Part I - Molecular Mechanisms of Activation and Regulation.
  Journal
Front Immunol 6:262 (2015)
DOI:10.3389/fimmu.2015.00262
Reference
  Authors
Oikonomopoulou K, Ricklin D, Ward PA, Lambris JD
  Title
Interactions between coagulation and complement--their role in inflammation.
  Journal
Semin Immunopathol 34:151-65 (2012)
DOI:10.1007/s00281-011-0280-x
Reference
  Authors
Kurosawa S, Stearns-Kurosawa DJ
  Title
Complement, thrombotic microangiopathy and disseminated intravascular coagulation.
  Journal
J Intensive Care 2:65 (2014)
DOI:10.1186/s40560-014-0061-4
Reference
  Authors
Hillmeister P, Persson PB
  Title
The Kallikrein-Kinin system.
  Journal
Acta Physiol (Oxf) 206:215-9 (2012)
DOI:10.1111/apha.12007
Reference
  Authors
Rau JC, Beaulieu LM, Huntington JA, Church FC
  Title
Serpins in thrombosis, hemostasis and fibrinolysis.
  Journal
J Thromb Haemost 5 Suppl 1:102-15 (2007)
DOI:10.1111/j.1538-7836.2007.02516.x
Reference
  Authors
Crooks MG, Hart SP
  Title
Coagulation and anticoagulation in idiopathic pulmonary fibrosis.
  Journal
Eur Respir Rev 24:392-9 (2015)
DOI:10.1183/16000617.00008414
Reference
  Authors
Christiaans SC, Wagener BM, Esmon CT, Pittet JF
  Title
Protein C and acute inflammation: a clinical and biological perspective.
  Journal
Am J Physiol Lung Cell Mol Physiol 305:L455-66 (2013)
DOI:10.1152/ajplung.00093.2013
Reference
  Authors
Alberelli MA, De Candia E
  Title
Functional role of protease activated receptors in vascular biology.
  Journal
Vascul Pharmacol 62:72-81 (2014)
DOI:10.1016/j.vph.2014.06.001
Reference
  Authors
Smith HW, Marshall CJ
  Title
Regulation of cell signalling by uPAR.
  Journal
Nat Rev Mol Cell Biol 11:23-36 (2010)
DOI:10.1038/nrm2821
Reference
  Authors
Ricklin D, Lambris JD
  Title
Complement in immune and inflammatory disorders: pathophysiological mechanisms.
  Journal
J Immunol 190:3831-8 (2013)
DOI:10.4049/jimmunol.1203487
Reference
  Authors
Ricklin D, Lambris JD
  Title
Complement in immune and inflammatory disorders: therapeutic interventions.
  Journal
J Immunol 190:3839-47 (2013)
DOI:10.4049/jimmunol.1203200
Reference
  Authors
Loof TG, Deicke C, Medina E
  Title
The role of coagulation/fibrinolysis during Streptococcus pyogenes infection.
  Journal
Front Cell Infect Microbiol 4:128 (2014)
DOI:10.3389/fcimb.2014.00128
Reference
  Authors
Jozsi M, Tortajada A, Uzonyi B, Goicoechea de Jorge E, Rodriguez de Cordoba S
  Title
Factor H-related proteins determine complement-activating surfaces.
  Journal
Trends Immunol 36:374-84 (2015)
DOI:10.1016/j.it.2015.04.008
Related
pathway
tfn04611  Platelet activation
tfn04662  B cell receptor signaling pathway
tfn04750  Inflammatory mediator regulation of TRP channels
KO pathway
ko04610   
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