KEGG   PATHWAY: gvr04610
Entry
gvr04610                    Pathway                                
Name
Complement and coagulation cascades - Galeopterus variegatus (Sunda flying lemur)
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
gvr04610  Complement and coagulation cascades
gvr04610

Organism
Galeopterus variegatus (Sunda flying lemur) [GN:gvr]
Gene
103581445  KLKB1; plasma kallikrein [KO:K01324] [EC:3.4.21.34]
103582723  [KO:K04012]
103583189  [KO:K04010]
103583874  [KO:K03991]
103584149  C3AR1; C3a anaphylatoxin chemotactic receptor [KO:K04009]
103584260  [KO:K01344] [EC:3.4.21.69]
103584903  SERPINF2; alpha-2-antiplasmin [KO:K03983]
103587636  PROS1; vitamin K-dependent protein S [KO:K03908]
103588297  F13A1; coagulation factor XIII A chain [KO:K03917] [EC:2.3.2.13]
103589069  PLAUR; urokinase plasminogen activator surface receptor [KO:K03985]
103589268  PLAT; tissue-type plasminogen activator [KO:K01343] [EC:3.4.21.68]
103589566  PROCR; endothelial protein C receptor [KO:K06557]
103589907  [KO:K01333] [EC:3.4.21.45]
103590492  C1QA; complement C1q subcomponent subunit A [KO:K03986]
103590497  C1QC; complement C1q subcomponent subunit C [KO:K03988]
103590523  C1QB; complement C1q subcomponent subunit B [KO:K03987]
103590694  TFPI; tissue factor pathway inhibitor [KO:K03909]
103591011  C5; complement C5 [KO:K03994]
103591826  PROC; vitamin K-dependent protein C [KO:K01344] [EC:3.4.21.69]
103591895  CPB2; carboxypeptidase B2 isoform X1 [KO:K01300] [EC:3.4.17.20]
103592016  PLG; plasminogen [KO:K01315] [EC:3.4.21.7]
103592060  FGB; fibrinogen beta chain [KO:K03904]
103592061  FGA; fibrinogen alpha chain [KO:K03903]
103592062  FGG; fibrinogen gamma chain isoform X1 [KO:K03905]
103592343  C4A; complement C4-A isoform X1 [KO:K03989]
103592349  C2; complement C2 [KO:K01332] [EC:3.4.21.43]
103592350  CFB; complement factor B [KO:K01335] [EC:3.4.21.47]
103594368  F2RL3; proteinase-activated receptor 4 [KO:K04236]
103594496  SERPINE2; glia-derived nexin [KO:K16643]
103594909  F12; coagulation factor XII [KO:K01328] [EC:3.4.21.38]
103594974  SERPINC1; antithrombin-III [KO:K03911]
103595134  PLAU; urokinase-type plasminogen activator [KO:K01348] [EC:3.4.21.73]
103595337  F2RL2; proteinase-activated receptor 3 [KO:K04235]
103595616  SERPINB2; LOW QUALITY PROTEIN: plasminogen activator inhibitor 2 [KO:K19821]
103595775  C1R; complement C1r subcomponent [KO:K01330] [EC:3.4.21.41]
103595776  C1S; complement C1s subcomponent [KO:K01331] [EC:3.4.21.42]
103596046  CD46; membrane cofactor protein isoform X1 [KO:K04007]
103596051  [KO:K04011]
103596198  VTN; vitronectin [KO:K06251]
103596324  SERPING1; plasma protease C1 inhibitor [KO:K04001]
103597281  MBL2; mannose-binding protein C [KO:K03991]
103597654  C6; complement component C6 [KO:K03995]
103598676  KNG1; kininogen-1 isoform X1 [KO:K03898]
103598974  F10; coagulation factor X [KO:K01314] [EC:3.4.21.6]
103598975  F7; coagulation factor VII [KO:K01320] [EC:3.4.21.21]
103599334  ITGB2; integrin beta-2 [KO:K06464]
103599528  C3; complement C3 [KO:K03990]
103599995  MASP2; mannan-binding lectin serine protease 2 [KO:K03993] [EC:3.4.21.104]
103600071  C7; complement component C7 [KO:K03996]
103600077  VWF; von Willebrand factor [KO:K03900]
103600149  F9; coagulation factor IX [KO:K01321] [EC:3.4.21.22]
103603178  C4BPA; C4b-binding protein alpha chain [KO:K04002]
103603179  C4BPB; C4b-binding protein beta chain isoform X1 [KO:K04003]
103603310  THBD; thrombomodulin [KO:K03907]
103603381  F2; prothrombin [KO:K01313] [EC:3.4.21.5]
103603475  CD59; CD59 glycoprotein [KO:K04008]
103603481  VSIG4; LOW QUALITY PROTEIN: V-set and immunoglobulin domain-containing protein 4 [KO:K19822]
103603505  SERPIND1; heparin cofactor 2 isoform X1 [KO:K03912]
103603579  C8B; LOW QUALITY PROTEIN: complement component C8 beta chain [KO:K03998]
103603589  C8A; LOW QUALITY PROTEIN: complement component C8 alpha chain [KO:K03997]
103603615  SERPINA5; plasma serine protease inhibitor [KO:K03913]
103604130  F2R; LOW QUALITY PROTEIN: proteinase-activated receptor 1 [KO:K03914]
103604901  SERPINE1; plasminogen activator inhibitor 1 isoform X1 [KO:K03982]
103605015  A2M; alpha-2-macroglobulin [KO:K03910]
103605330  F3; tissue factor [KO:K03901]
103605607  [KO:K03991]
103605884  BDKRB1; B1 bradykinin receptor [KO:K03915]
103606496  CFH; complement factor H [KO:K04004]
103606680  MASP1; mannan-binding lectin serine protease 1 [KO:K03992] [EC:3.4.21.-]
103606839  CLU; clusterin [KO:K17252]
103607430  F13B; coagulation factor XIII B chain [KO:K03906]
103607668  CFD; complement factor D [KO:K01334] [EC:3.4.21.46]
103608021  [KO:K03899]
103608542  [KO:K03984]
103608802  ITGAX; integrin alpha-X [KO:K06462]
103609109  [KO:K04006]
103609598  [KO:K04010]
103609637  F5; coagulation factor V [KO:K03902]
103610123  [KO:K03914]
103610124  ITGAM; integrin alpha-M [KO:K06461]
103610433  [KO:K04002]
103610748  BDKRB2; B2 bradykinin receptor [KO:K03916]
103610902  [KO:K04012]
103610990  C8G; LOW QUALITY PROTEIN: complement component C8 gamma chain [KO:K03999]
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
gvr04611  Platelet activation
gvr04662  B cell receptor signaling pathway
gvr04750  Inflammatory mediator regulation of TRP channels
KO pathway
ko04610   

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