KEGG   PATHWAY: oor04610
Entry
oor04610                    Pathway                                
Name
Complement and coagulation cascades - Orcinus orca (killer whale)
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
oor04610  Complement and coagulation cascades
oor04610

Organism
Orcinus orca (killer whale) [GN:oor]
Gene
101277688  F7; coagulation factor VII isoform X1 [KO:K01320] [EC:3.4.21.21]
101274877  F3; tissue factor [KO:K03901]
101286772  F12; LOW QUALITY PROTEIN: coagulation factor XII [KO:K01328] [EC:3.4.21.38]
101278094  coagulation factor XI isoform X1 [KO:K01323] [EC:3.4.21.27]
101271539  F9; coagulation factor IX [KO:K01321] [EC:3.4.21.22]
101277710  VWF; von Willebrand factor isoform X1 [KO:K03900]
101284644  F8; coagulation factor VIII [KO:K03899]
101275181  F10; coagulation factor X [KO:K01314] [EC:3.4.21.6]
101290204  F5; coagulation factor V isoform X1 [KO:K03902]
101289120  F2; prothrombin [KO:K01313] [EC:3.4.21.5]
101277878  FGA; fibrinogen alpha chain [KO:K03903]
101277474  FGB; fibrinogen beta chain isoform X2 [KO:K03904]
101278122  FGG; fibrinogen gamma chain isoform X1 [KO:K03905]
101286489  F13A1; coagulation factor XIII A chain [KO:K03917] [EC:2.3.2.13]
101270996  F13B; coagulation factor XIII B chain isoform X2 [KO:K03906]
101278325  F2R; proteinase-activated receptor 1 isoform X1 [KO:K03914]
101278076  F2RL2; proteinase-activated receptor 3 [KO:K04235]
101269646  F2RL3; proteinase-activated receptor 4 [KO:K04236]
101269719  CPB2; carboxypeptidase B2 [KO:K01300] [EC:3.4.17.20]
101284582  THBD; thrombomodulin [KO:K03907]
101283592  PROCR; endothelial protein C receptor [KO:K06557]
101270550  PROC; vitamin K-dependent protein C [KO:K01344] [EC:3.4.21.69]
101269959  PROS1; vitamin K-dependent protein S [KO:K03908]
101273613  SERPINE1; plasminogen activator inhibitor 1 [KO:K03982]
101270776  plasminogen activator inhibitor 2 [KO:K19821]
101274868  SERPINA5; plasma serine protease inhibitor isoform X2 [KO:K03913]
101273487  SERPINE2; glia-derived nexin [KO:K16643]
101285324  PLAT; tissue-type plasminogen activator isoform X1 [KO:K01343] [EC:3.4.21.68]
101281249  PLAU; urokinase-type plasminogen activator isoform X3 [KO:K01348] [EC:3.4.21.73]
101276007  PLAUR; urokinase plasminogen activator surface receptor isoform X1 [KO:K03985]
101277612  PLG; plasminogen [KO:K01315] [EC:3.4.21.7]
101283768  KNG1; kininogen-1 isoform X1 [KO:K03898]
101277133  BDKRB1; B1 bradykinin receptor [KO:K03915]
101271639  BDKRB2; B2 bradykinin receptor [KO:K03916]
101287826  TFPI; tissue factor pathway inhibitor isoform X1 [KO:K03909]
101273903  SERPINA1; alpha-1-antitrypsin [KO:K03984]
101274588  SERPINC1; antithrombin-III isoform X2 [KO:K03911]
101271283  SERPIND1; heparin cofactor 2 [KO:K03912]
101280186  SERPING1; plasma protease C1 inhibitor isoform X2 [KO:K04001]
101280554  SERPINF2; alpha-2-antiplasmin [KO:K03983]
101271303  A2M; alpha-2-macroglobulin isoform X1 [KO:K03910]
101269650  C1R; complement C1r subcomponent isoform X2 [KO:K01330] [EC:3.4.21.41]
101269409  C1S; complement C1s subcomponent [KO:K01331] [EC:3.4.21.42]
101284231  MBL2; LOW QUALITY PROTEIN: mannose-binding protein C [KO:K03991]
101289647  mannose-binding protein A-like [KO:K03991]
101285880  MASP1; mannan-binding lectin serine protease 1 isoform X1 [KO:K03992] [EC:3.4.21.-]
101283337  MASP2; mannan-binding lectin serine protease 2 isoform X2 [KO:K03993] [EC:3.4.21.104]
101279376  complement C4 isoform X1 [KO:K03989]
101281566  C2; complement C2 [KO:K01332] [EC:3.4.21.43]
101281425  C3; complement C3 [KO:K03990]
101281312  CFB; complement factor B [KO:K01335] [EC:3.4.21.47]
101272668  CFD; complement factor D [KO:K01334] [EC:3.4.21.46]
101279383  C5; complement C5 isoform X1 [KO:K03994]
101286425  C6; complement component C6 [KO:K03995]
101286676  C7; complement component C7 isoform X1 [KO:K03996]
101290142  C8A; complement component C8 alpha chain isoform X1 [KO:K03997]
101289903  C8B; complement component C8 beta chain [KO:K03998]
101276914  C8G; complement component C8 gamma chain [KO:K03999]
101289460  C9; complement component C9 isoform X1 [KO:K04000]
101272036  C3AR1; C3a anaphylatoxin chemotactic receptor isoform X2 [KO:K04009]
101275222  VSIG4; V-set and immunoglobulin domain-containing protein 4 isoform X1 [KO:K19822]
101290167  CR1; complement receptor type 1 isoform X1 [KO:K04011]
101269842  CR2; complement receptor type 2 isoform X3 [KO:K04012]
101287801  ITGAM; integrin alpha-M isoform X1 [KO:K06461]
101285903  ITGB2; integrin beta-2 isoform X3 [KO:K06464]
101288051  integrin alpha-X isoform X1 [KO:K06462]
101270207  C5AR1; C5a anaphylatoxin chemotactic receptor 1 [KO:K04010]
101269843  C4BPA; C4b-binding protein alpha chain isoform X1 [KO:K04002]
125960240  C4BPB; C4b-binding protein beta chain isoform X1 [KO:K04003]
101289802  CFI; complement factor I isoform X1 [KO:K01333] [EC:3.4.21.45]
101271984  complement factor H [KO:K04004]
101289924  CD46; membrane cofactor protein isoform X3 [KO:K04007]
117197676  membrane cofactor protein-like isoform X1 [KO:K04007]
101269344  CD55; complement decay-accelerating factor isoform X2 [KO:K04006]
101270585  CD59; CD59 glycoprotein [KO:K04008]
101270919  LOW QUALITY PROTEIN: CD59 glycoprotein [KO:K04008]
101282975  CLU; clusterin [KO:K17252]
101278304  VTN; vitronectin isoform X1 [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
oor04611  Platelet activation
oor04662  B cell receptor signaling pathway
oor04750  Inflammatory mediator regulation of TRP channels
KO pathway
ko04610   
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