KEGG   PATHWAY: nle04610
Entry
nle04610                    Pathway                                
Name
Complement and coagulation cascades - Nomascus leucogenys (northern white-cheeked gibbon)
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
nle04610  Complement and coagulation cascades
nle04610

Organism
Nomascus leucogenys (northern white-cheeked gibbon) [GN:nle]
Gene
100607316  F7; LOW QUALITY PROTEIN: coagulation factor VII [KO:K01320] [EC:3.4.21.21]
100596603  F3; tissue factor [KO:K03901]
100586368  F12; coagulation factor XII [KO:K01328] [EC:3.4.21.38]
100594390  F11; coagulation factor XI [KO:K01323] [EC:3.4.21.27]
100598601  F9; coagulation factor IX isoform X1 [KO:K01321] [EC:3.4.21.22]
100580759  VWF; von Willebrand factor [KO:K03900]
100606287  F8; coagulation factor VIII isoform X1 [KO:K03899]
100607918  F10; coagulation factor X isoform X1 [KO:K01314] [EC:3.4.21.6]
100582223  F5; coagulation factor V [KO:K03902]
100595467  F2; prothrombin [KO:K01313] [EC:3.4.21.5]
100607544  FGA; fibrinogen alpha chain [KO:K03903]
100606953  FGB; fibrinogen beta chain isoform X1 [KO:K03904]
100579325  FGG; fibrinogen gamma chain isoform X1 [KO:K03905]
100581513  F13A1; coagulation factor XIII A chain [KO:K03917] [EC:2.3.2.13]
100600587  F13B; coagulation factor XIII B chain [KO:K03906]
100607663  F2R; proteinase-activated receptor 1 [KO:K03914]
100607317  F2RL2; proteinase-activated receptor 3 [KO:K04235]
100597937  F2RL3; proteinase-activated receptor 4 [KO:K04236]
100582393  CPB2; carboxypeptidase B2 isoform X1 [KO:K01300] [EC:3.4.17.20]
100600139  THBD; thrombomodulin [KO:K03907]
100606588  PROCR; endothelial protein C receptor isoform X1 [KO:K06557]
100602687  PROC; vitamin K-dependent protein C isoform X1 [KO:K01344] [EC:3.4.21.69]
100600356  vitamin K-dependent protein S [KO:K03908]
100588010  SERPINE1; plasminogen activator inhibitor 1 isoform X1 [KO:K03982]
100603986  SERPINB2; plasminogen activator inhibitor 2 [KO:K19821]
100586470  SERPINA5; plasma serine protease inhibitor isoform X2 [KO:K03913]
100596009  SERPINE2; glia-derived nexin isoform X1 [KO:K16643]
100586515  PLAT; tissue-type plasminogen activator [KO:K01343] [EC:3.4.21.68]
100581844  PLAU; urokinase-type plasminogen activator [KO:K01348] [EC:3.4.21.73]
100581691  PLAUR; urokinase plasminogen activator surface receptor isoform X1 [KO:K03985]
101177406  PLG; plasminogen [KO:K01315] [EC:3.4.21.7]
100594044  plasma kallikrein isoform X1 [KO:K01324] [EC:3.4.21.34]
115835879  plasma kallikrein-like [KO:K01324] [EC:3.4.21.34]
100597381  KNG1; kininogen-1 isoform X2 [KO:K03898]
100599756  BDKRB1; B1 bradykinin receptor [KO:K03915]
100599411  BDKRB2; B2 bradykinin receptor [KO:K03916]
100604816  TFPI; tissue factor pathway inhibitor isoform X3 [KO:K03909]
100585221  SERPINA1; alpha-1-antitrypsin isoform X3 [KO:K03984]
100603948  SERPINC1; antithrombin-III isoform X1 [KO:K03911]
100596966  SERPIND1; heparin cofactor 2 isoform X2 [KO:K03912]
100583544  SERPING1; plasma protease C1 inhibitor isoform X2 [KO:K04001]
100585210  SERPINF2; alpha-2-antiplasmin isoform X1 [KO:K03983]
100595735  alpha-2-macroglobulin isoform X2 [KO:K03910]
100606692  C1QA; complement C1q subcomponent subunit A [KO:K03986]
100607840  C1QB; complement C1q subcomponent subunit B [KO:K03987]
100607041  C1QC; complement C1q subcomponent subunit C isoform X1 [KO:K03988]
100591142  C1R; complement C1r subcomponent [KO:K01330] [EC:3.4.21.41]
100599399  C1S; complement C1s subcomponent isoform X1 [KO:K01331] [EC:3.4.21.42]
100597029  MBL2; mannose-binding protein C [KO:K03991]
100590227  mannose-binding protein A-like [KO:K03991]
100601109  MASP1; mannan-binding lectin serine protease 1 isoform X1 [KO:K03992] [EC:3.4.21.-]
100593253  MASP2; mannan-binding lectin serine protease 2 isoform X1 [KO:K03993] [EC:3.4.21.104]
100596445  complement C4-B isoform X2 [KO:K03989]
100606928  C2; complement C2 isoform X1 [KO:K01332] [EC:3.4.21.43]
100598784  C3; complement C3 [KO:K03990]
100595765  CFB; complement factor B isoform X3 [KO:K01335] [EC:3.4.21.47]
100599422  CFD; complement factor D [KO:K01334] [EC:3.4.21.46]
100606437  C5; complement C5 isoform X2 [KO:K03994]
100598505  C6; complement component C6 isoform X2 [KO:K03995]
100598163  C7; complement component C7 [KO:K03996]
100582717  C8A; complement component C8 alpha chain [KO:K03997]
100583059  C8B; complement component C8 beta chain isoform X2 [KO:K03998]
100605861  C8G; complement component C8 gamma chain isoform X2 [KO:K03999]
100593494  C9; complement component C9 [KO:K04000]
100588722  C3AR1; C3a anaphylatoxin chemotactic receptor [KO:K04009]
100602994  VSIG4; V-set and immunoglobulin domain-containing protein 4 isoform X1 [KO:K19822]
100587880  CR1; LOW QUALITY PROTEIN: complement receptor type 1 [KO:K04011]
100587321  CR2; complement receptor type 2 isoform X2 [KO:K04012]
100603969  ITGAM; integrin alpha-M [KO:K06461]
100600337  ITGB2; integrin beta-2 isoform X1 [KO:K06464]
100595603  ITGAX; integrin alpha-X [KO:K06462]
100589337  C5AR1; C5a anaphylatoxin chemotactic receptor 1 [KO:K04010]
100587775  C4BPA; C4b-binding protein alpha chain [KO:K04002]
100588098  LOW QUALITY PROTEIN: C4b-binding protein alpha chain-like [KO:K04002]
100582942  C4BPB; C4b-binding protein beta chain isoform X1 [KO:K04003]
100590769  CFI; complement factor I [KO:K01333] [EC:3.4.21.45]
100600041  CFH; complement factor H isoform X1 [KO:K04004]
100588200  CD46; membrane cofactor protein isoform X7 [KO:K04007]
100586537  CD55; complement decay-accelerating factor isoform X3 [KO:K04006]
100599698  complement factor H-related protein 2 isoform X1 [KO:K23815]
100586937  LOW QUALITY PROTEIN: complement factor H-related protein 4-like [KO:K23816]
100599128  CFHR5; complement factor H-related protein 5 isoform X1 [KO:K23817]
100579645  CD59; CD59 glycoprotein [KO:K04008]
100586861  CLU; clusterin [KO:K17252]
100592274  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
nle04611  Platelet activation
nle04662  B cell receptor signaling pathway
nle04750  Inflammatory mediator regulation of TRP channels
KO pathway
ko04610   
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