KEGG   PATHWAY: mcf04610
Entry
mcf04610                    Pathway                                
Name
Complement and coagulation cascades - Macaca fascicularis (crab-eating macaque)
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
mcf04610  Complement and coagulation cascades
mcf04610

Organism
Macaca fascicularis (crab-eating macaque) [GN:mcf]
Gene
101865088  PROS1; vitamin K-dependent protein S isoform X1 [KO:K03908]
101865984  SERPINA5; plasma serine protease inhibitor [KO:K03913]
101866035  ITGAM; integrin alpha-M isoform X1 [KO:K06461]
101866354  [KO:K01330] [EC:3.4.21.41]
101866929  CFI; complement factor I isoform X1 [KO:K01333] [EC:3.4.21.45]
101866962  MASP1; mannan-binding lectin serine protease 1 isoform X2 [KO:K03992] [EC:3.4.21.-]
101867071  CD59; CD59 glycoprotein isoform X3 [KO:K04008]
101925124  ITGB2; integrin beta-2 isoform X1 [KO:K06464]
101925781  F12; coagulation factor XII [KO:K01328] [EC:3.4.21.38]
101926212  [KO:K04002]
102116032  C6; complement component C6 [KO:K03995]
102116557  SERPINF2; alpha-2-antiplasmin isoform X1 [KO:K03983]
102116805  CR1L; complement component receptor 1-like protein isoform X1 [KO:K04011]
102117109  F2RL3; proteinase-activated receptor 4 isoform X2 [KO:K04236]
102117401  CR1; complement receptor type 1 isoform X1 [KO:K04011]
102117641  SERPINC1; antithrombin-III [KO:K03911]
102118738  FGB; fibrinogen beta chain [KO:K03904]
102118947  C3AR1; C3a anaphylatoxin chemotactic receptor [KO:K04009]
102119374  FGA; fibrinogen alpha chain [KO:K03903]
102119426  [KO:K03991]
102119734  TFPI; tissue factor pathway inhibitor isoform X2 [KO:K03909]
102119751  FGG; fibrinogen gamma chain [KO:K03905]
102120463  SERPIND1; heparin cofactor 2 [KO:K03912]
102120567  MBL2; mannose-binding protein C isoform X1 [KO:K03991]
102121077  F7; coagulation factor VII isoform X1 [KO:K01320] [EC:3.4.21.21]
102122506  C5AR1; C5a anaphylatoxin chemotactic receptor 1 isoform X1 [KO:K04010]
102123453  BDKRB1; B1 bradykinin receptor [KO:K03915]
102123546  [KO:K03989]
102123736  F10; coagulation factor X [KO:K01314] [EC:3.4.21.6]
102124671  KNG1; kininogen-1 [KO:K03898]
102125064  THBD; thrombomodulin [KO:K03907]
102125498  F8; coagulation factor VIII isoform X1 [KO:K03899]
102125658  C5; complement C5 isoform X1 [KO:K03994]
102126128  F13A1; coagulation factor XIII A chain isoform X1 [KO:K03917] [EC:2.3.2.13]
102126692  C8G; complement component C8 gamma chain [KO:K03999]
102130549  C1QC; complement C1q subcomponent subunit C isoform X1 [KO:K03988]
102130704  SERPINB2; plasminogen activator inhibitor 2 [KO:K19821]
102130734  A2M; alpha-2-macroglobulin [KO:K03910]
102130928  VTN; vitronectin isoform X1 [KO:K06251]
102131020  F2; prothrombin [KO:K01313] [EC:3.4.21.5]
102131431  F3; tissue factor isoform X1 [KO:K03901]
102131458  C3; complement C3 [KO:K03990]
102132156  C4B; complement C4-B [KO:K03989]
102132600  CPB2; carboxypeptidase B2 isoform X1 [KO:K01300] [EC:3.4.17.20]
102132631  VWF; von Willebrand factor isoform X1 [KO:K03900]
102133338  C8B; complement component C8 beta chain isoform X1 [KO:K03998]
102133702  CFH; complement factor H isoform X1 [KO:K04004]
102133722  C8A; complement component C8 alpha chain isoform X1 [KO:K03997]
102133787  CFB; complement factor B [KO:K01335] [EC:3.4.21.47]
102134603  CFHR2; complement factor H-related protein 2 isoform X1 [KO:K23815]
102134961  SERPING1; plasma protease C1 inhibitor [KO:K04001]
102134991  SERPINA1; alpha-1-antitrypsin isoform X2 [KO:K03984]
102135038  [KO:K01330] [EC:3.4.21.41]
102135397  PROCR; endothelial protein C receptor [KO:K06557]
102135466  CFHR5; complement factor H-related protein 5 [KO:K23817]
102135485  CLU; clusterin isoform X1 [KO:K17252]
102135886  PLAU; urokinase-type plasminogen activator isoform X1 [KO:K01348] [EC:3.4.21.73]
102136100  F2RL2; proteinase-activated receptor 3 isoform X1 [KO:K04235]
102136210  PROC; vitamin K-dependent protein C isoform X5 [KO:K01344] [EC:3.4.21.69]
102136369  F13B; coagulation factor XIII B chain isoform X1 [KO:K03906]
102136571  CFD; complement factor D isoform X1 [KO:K01334] [EC:3.4.21.46]
102137096  C2; complement C2 [KO:K01332] [EC:3.4.21.43]
102137130  F2R; proteinase-activated receptor 1 [KO:K03914]
102137207  C1S; complement C1s subcomponent [KO:K01331] [EC:3.4.21.42]
102137382  C9; complement component C9 [KO:K04000]
102139334  PLAUR; urokinase plasminogen activator surface receptor isoform X1 [KO:K03985]
102139374  [KO:K23816]
102139434  C1QB; complement C1q subcomponent subunit B [KO:K03987]
102140557  C4BPB; C4b-binding protein beta chain isoform X1 [KO:K04003]
102141482  F5; coagulation factor V [KO:K03902]
102142263  C4BPA; C4b-binding protein alpha chain isoform X1 [KO:K04002]
102142369  KLKB1; plasma kallikrein [KO:K01324] [EC:3.4.21.34]
102143404  SERPINE2; glia-derived nexin isoform X1 [KO:K16643]
102143421  F11; coagulation factor XI isoform X1 [KO:K01323] [EC:3.4.21.27]
102143442  CD55; complement decay-accelerating factor isoform X5 [KO:K04006]
102144784  VSIG4; V-set and immunoglobulin domain-containing protein 4 isoform X1 [KO:K19822]
102145462  PLAT; tissue-type plasminogen activator [KO:K01343] [EC:3.4.21.68]
102145559  CR2; complement receptor type 2 isoform X1 [KO:K04012]
102145643  PLG; plasminogen [KO:K01315] [EC:3.4.21.7]
102146175  F9; coagulation factor IX [KO:K01321] [EC:3.4.21.22]
102146832  CD46; membrane cofactor protein isoform X1 [KO:K04007]
102147003  SERPINE1; plasminogen activator inhibitor 1 [KO:K03982]
102147055  C7; complement component C7 [KO:K03996]
102147272  ITGAX; integrin alpha-X isoform X1 [KO:K06462]
107126378  BDKRB2; B2 bradykinin receptor [KO:K03916]
107126479  C1QA; complement C1q subcomponent subunit A [KO:K03986]
135966149  [KO:K01331] [EC:3.4.21.42]
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
mcf04611  Platelet activation
mcf04662  B cell receptor signaling pathway
mcf04750  Inflammatory mediator regulation of TRP channels
KO pathway
ko04610   

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