KEGG   PATHWAY: pon04610
Entry
pon04610                    Pathway                                
Name
Complement and coagulation cascades - Pongo abelii (Sumatran orangutan)
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
pon04610  Complement and coagulation cascades
pon04610

Organism
Pongo abelii (Sumatran orangutan) [GN:pon]
Gene
100452636  F7; LOW QUALITY PROTEIN: coagulation factor VII [KO:K01320] [EC:3.4.21.21]
100173124  F3; tissue factor precursor [KO:K03901]
100434389  F12; coagulation factor XII [KO:K01328] [EC:3.4.21.38]
100443368  F11; coagulation factor XI [KO:K01323] [EC:3.4.21.27]
100446459  F9; coagulation factor IX isoform X1 [KO:K01321] [EC:3.4.21.22]
100434817  VWF; von Willebrand factor precursor [KO:K03900]
100174452  F8; coagulation factor VIII isoform X1 [KO:K03899]
100453258  F10; coagulation factor X isoform X1 [KO:K01314] [EC:3.4.21.6]
100446389  F5; coagulation factor V [KO:K03902]
100173859  F2; prothrombin precursor [KO:K01313] [EC:3.4.21.5]
100461464  FGA; fibrinogen alpha chain isoform X1 [KO:K03903]
100173861  FGB; fibrinogen beta chain precursor [KO:K03904]
100461832  FGG; fibrinogen gamma chain isoform X2 [KO:K03905]
100440931  F13A1; coagulation factor XIII A chain isoform X1 [KO:K03917] [EC:2.3.2.13]
100450158  F13B; coagulation factor XIII B chain [KO:K03906]
100447792  F2R; proteinase-activated receptor 1 [KO:K03914]
100447178  F2RL2; proteinase-activated receptor 3 isoform X1 [KO:K04235]
100447897  F2RL3; proteinase-activated receptor 4 [KO:K04236]
100452501  CPB2; carboxypeptidase B2 isoform X1 [KO:K01300] [EC:3.4.17.20]
100443362  THBD; thrombomodulin [KO:K03907]
100456578  PROCR; endothelial protein C receptor isoform X1 [KO:K06557]
100448857  PROC; vitamin K-dependent protein C isoform X1 [KO:K01344] [EC:3.4.21.69]
100458135  PROS1; vitamin K-dependent protein S isoform X1 [KO:K03908]
100435876  SERPINE1; LOW QUALITY PROTEIN: plasminogen activator inhibitor 1 [KO:K03982]
103893003  SERPINB2; plasminogen activator inhibitor 2 [KO:K19821]
100459205  SERPINA5; plasma serine protease inhibitor [KO:K03913]
100439307  SERPINE2; glia-derived nexin [KO:K16643]
100173068  PLAT; tissue-type plasminogen activator precursor [KO:K01343] [EC:3.4.21.68]
100171578  PLAU; urokinase-type plasminogen activator precursor [KO:K01348] [EC:3.4.21.73]
100461710  PLAUR; urokinase plasminogen activator surface receptor isoform X1 [KO:K03985]
100172984  PLG; plasminogen precursor [KO:K01315] [EC:3.4.21.7]
100172926  KLKB1; plasma kallikrein [KO:K01324] [EC:3.4.21.34]
100432100  KNG1; kininogen-1 isoform X1 [KO:K03898]
100434593  BDKRB1; B1 bradykinin receptor isoform X2 [KO:K03915]
100434213  BDKRB2; B2 bradykinin receptor isoform X1 [KO:K03916]
100171528  TFPI; tissue factor pathway inhibitor precursor [KO:K03909]
100171544  SERPINA1; alpha-1-antitrypsin precursor [KO:K03984]
100173818  SERPINC1; antithrombin-III precursor [KO:K03911]
100442934  SERPIND1; heparin cofactor 2 isoform X2 [KO:K03912]
100173828  SERPING1; plasma protease C1 inhibitor precursor [KO:K04001]
100457028  SERPINF2; alpha-2-antiplasmin isoform X2 [KO:K03983]
100173946  A2M; alpha-2-macroglobulin precursor [KO:K03910]
100433850  C1QA; complement C1q subcomponent subunit A isoform X2 [KO:K03986]
100435212  C1QB; complement C1q subcomponent subunit B isoform X1 [KO:K03987]
100432946  C1QC; complement C1q subcomponent subunit C isoform X1 [KO:K03988]
100173855  C1R; complement C1r subcomponent precursor [KO:K01330] [EC:3.4.21.41]
112135770  complement C1r subcomponent-like [KO:K01330] [EC:3.4.21.41]
100455685  C1S; complement C1s subcomponent [KO:K01331] [EC:3.4.21.42]
100432027  LOW QUALITY PROTEIN: complement C1s subcomponent-like [KO:K01331] [EC:3.4.21.42]
100434809  MBL2; mannose-binding protein C [KO:K03991]
100462002  LOW QUALITY PROTEIN: mannose-binding protein A-like [KO:K03991]
100439814  MASP1; mannan-binding lectin serine protease 1 isoform X4 [KO:K03992] [EC:3.4.21.-]
100458242  MASP2; mannan-binding lectin serine protease 2 isoform X2 [KO:K03993] [EC:3.4.21.104]
100171517  C4B; LOW QUALITY PROTEIN: complement C4-B [KO:K03989]
100173033  C2; complement C2 precursor [KO:K01332] [EC:3.4.21.43]
100446798  C3; complement C3 isoform X1 [KO:K03990]
103890868  CFB; complement factor B [KO:K01335] [EC:3.4.21.47]
100936423  CFD; complement factor D [KO:K01334] [EC:3.4.21.46]
100461390  C5; complement C5 [KO:K03994]
100447541  C6; complement component C6 isoform X1 [KO:K03995]
100172681  C7; complement component C7 precursor [KO:K03996]
100454371  C8A; complement component C8 alpha chain [KO:K03997]
100453748  C8B; complement component C8 beta chain isoform X3 [KO:K03998]
100459054  C8G; complement component C8 gamma chain [KO:K03999]
100441040  C9; complement component C9 [KO:K04000]
100171704  C3AR1; C3a anaphylatoxin chemotactic receptor [KO:K04009]
100171405  VSIG4; V-set and immunoglobulin domain-containing protein 4 precursor [KO:K19822]
100443325  CR1; complement receptor type 1 [KO:K04011]
100438675  CR1L; complement component receptor 1-like protein [KO:K04011]
103890063  CR2; complement receptor type 2 isoform X1 [KO:K04012]
100439660  ITGAM; integrin alpha-M isoform X1 [KO:K06461]
100451938  ITGB2; integrin beta-2 [KO:K06464]
100440032  ITGAX; LOW QUALITY PROTEIN: integrin alpha-X [KO:K06462]
100455789  C5AR1; C5a anaphylatoxin chemotactic receptor 1 isoform X3 [KO:K04010]
100440751  C4BPA; C4b-binding protein alpha chain [KO:K04002]
100443687  zona pellucida sperm-binding protein 3 receptor-like [KO:K04002]
100441362  C4BPB; C4b-binding protein beta chain isoform X1 [KO:K04003]
100189676  CFI; complement factor I [KO:K01333] [EC:3.4.21.45]
100451270  CFH; complement factor H isoform X1 [KO:K04004]
100174740  CD46; membrane cofactor protein precursor [KO:K04007]
100439403  CD55; complement decay-accelerating factor isoform X2 [KO:K04006]
100450891  complement factor H-related protein 2 isoform X2 [KO:K23815]
103888513  CFHR2; complement factor H-related protein 2 isoform X1 [KO:K23815]
100451648  complement factor H-related protein 4 isoform X1 [KO:K23816]
100173870  CD59; CD59 glycoprotein precursor [KO:K04008]
100451729  CLU; clusterin isoform X2 [KO:K17252]
100174092  VTN; vitronectin precursor [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
pon04611  Platelet activation
pon04662  B cell receptor signaling pathway
pon04750  Inflammatory mediator regulation of TRP channels
KO pathway
ko04610   

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