KEGG   PATHWAY: pps04610
Entry
pps04610                    Pathway                                
Name
Complement and coagulation cascades - Pan paniscus (bonobo)
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
pps04610  Complement and coagulation cascades
pps04610

Organism
Pan paniscus (bonobo) [GN:pps]
Gene
100987335  F7; coagulation factor VII [KO:K01320] [EC:3.4.21.21]
100988955  F3; tissue factor isoform X1 [KO:K03901]
100995209  F12; coagulation factor XII isoform X1 [KO:K01328] [EC:3.4.21.38]
100986597  F11; coagulation factor XI isoform X2 [KO:K01323] [EC:3.4.21.27]
100969131  F9; coagulation factor IX [KO:K01321] [EC:3.4.21.22]
100985238  VWF; von Willebrand factor [KO:K03900]
100992135  F8; coagulation factor VIII isoform X1 [KO:K03899]
100987663  F10; coagulation factor X isoform X1 [KO:K01314] [EC:3.4.21.6]
100982913  F5; coagulation factor V [KO:K03902]
100975534  F2; prothrombin [KO:K01313] [EC:3.4.21.5]
100991875  FGA; fibrinogen alpha chain [KO:K03903]
100992445  FGB; fibrinogen beta chain [KO:K03904]
100991527  FGG; fibrinogen gamma chain isoform X2 [KO:K03905]
100982798  F13A1; coagulation factor XIII A chain [KO:K03917] [EC:2.3.2.13]
100967470  F13B; coagulation factor XIII B chain [KO:K03906]
100986369  F2R; proteinase-activated receptor 1 [KO:K03914]
100985507  F2RL2; proteinase-activated receptor 3 isoform X1 [KO:K04235]
100988111  F2RL3; proteinase-activated receptor 4 [KO:K04236]
100993828  CPB2; carboxypeptidase B2 [KO:K01300] [EC:3.4.17.20]
100971543  THBD; thrombomodulin [KO:K03907]
100981294  PROCR; endothelial protein C receptor isoform X1 [KO:K06557]
100972665  PROC; vitamin K-dependent protein C isoform X2 [KO:K01344] [EC:3.4.21.69]
100987890  LOW QUALITY PROTEIN: vitamin K-dependent protein S-like [KO:K03908]
100969680  PROS1; vitamin K-dependent protein S isoform X1 [KO:K03908]
100973457  SERPINE1; plasminogen activator inhibitor 1 [KO:K03982]
100994697  SERPINB2; plasminogen activator inhibitor 2 [KO:K19821]
100992073  SERPINA5; plasma serine protease inhibitor [KO:K03913]
100980533  SERPINE2; glia-derived nexin isoform X1 [KO:K16643]
100991098  PLAT; tissue-type plasminogen activator isoform X1 [KO:K01343] [EC:3.4.21.68]
100983615  PLAU; urokinase-type plasminogen activator [KO:K01348] [EC:3.4.21.73]
100980976  PLAUR; urokinase plasminogen activator surface receptor isoform X1 [KO:K03985]
100982214  plasminogen [KO:K01315] [EC:3.4.21.7]
100985593  KLKB1; plasma kallikrein isoform X1 [KO:K01324] [EC:3.4.21.34]
100995206  KNG1; kininogen-1 [KO:K03898]
100972709  BDKRB1; B1 bradykinin receptor [KO:K03915]
100972150  BDKRB2; B2 bradykinin receptor [KO:K03916]
100974935  TFPI; tissue factor pathway inhibitor isoform X1 [KO:K03909]
100990043  SERPINA1; alpha-1-antitrypsin [KO:K03984]
100975942  SERPINC1; antithrombin-III isoform X1 [KO:K03911]
100973104  SERPIND1; heparin cofactor 2 [KO:K03912]
100983502  plasma protease C1 inhibitor [KO:K04001]
100994793  SERPING1; plasma protease C1 inhibitor isoform X2 [KO:K04001]
100980053  SERPINF2; alpha-2-antiplasmin isoform X2 [KO:K03983]
100979269  alpha-2-macroglobulin isoform X1 [KO:K03910]
100993798  C1QA; complement C1q subcomponent subunit A [KO:K03986]
100994127  C1QB; complement C1q subcomponent subunit B [KO:K03987]
112440503  C1QC; complement C1q subcomponent subunit C [KO:K03988]
100991297  C1R; complement C1r subcomponent [KO:K01330] [EC:3.4.21.41]
100991971  C1S; complement C1s subcomponent [KO:K01331] [EC:3.4.21.42]
100977606  MBL2; mannose-binding protein C [KO:K03991]
100992380  mannose-binding protein A-like [KO:K03991]
100982840  mannose-binding protein A-like [KO:K03991]
100970830  MASP1; mannan-binding lectin serine protease 1 isoform X2 [KO:K03992] [EC:3.4.21.-]
100981899  MASP2; mannan-binding lectin serine protease 2 isoform X3 [KO:K03993] [EC:3.4.21.104]
100988452  complement C4-B [KO:K03989]
103783827  C2; complement C2 isoform X1 [KO:K01332] [EC:3.4.21.43]
100982432  C3; complement C3 [KO:K03990]
100990821  CFB; complement factor B [KO:K01335] [EC:3.4.21.47]
100986224  C5; complement C5 isoform X2 [KO:K03994]
100978093  C6; complement component C6 isoform X1 [KO:K03995]
100977761  C7; complement component C7 isoform X3 [KO:K03996]
100976288  C8A; complement component C8 alpha chain [KO:K03997]
100976626  C8B; complement component C8 beta chain [KO:K03998]
100982752  C8G; complement component C8 gamma chain isoform X2 [KO:K03999]
100974282  C9; complement component C9 isoform X1 [KO:K04000]
100990231  C3AR1; C3a anaphylatoxin chemotactic receptor [KO:K04009]
100978586  VSIG4; V-set and immunoglobulin domain-containing protein 4 isoform X1 [KO:K19822]
100982795  CR1; complement receptor type 1 isoform X1 [KO:K04011]
103783766  CR1L; LOW QUALITY PROTEIN: complement component receptor 1-like protein [KO:K04011]
100983119  CR2; complement receptor type 2 [KO:K04012]
100992002  ITGAM; integrin alpha-M isoform X1 [KO:K06461]
100985940  ITGB2; integrin beta-2 [KO:K06464]
100992342  ITGAX; integrin alpha-X [KO:K06462]
100975182  C5AR1; C5a anaphylatoxin chemotactic receptor 1 [KO:K04010]
100984709  C4BPA; C4b-binding protein alpha chain isoform X1 [KO:K04002]
100968163  LOW QUALITY PROTEIN: zona pellucida sperm-binding protein 3 receptor-like [KO:K04002]
100985346  C4BPB; C4b-binding protein beta chain isoform X3 [KO:K04003]
100972456  CFI; complement factor I isoform X3 [KO:K01333] [EC:3.4.21.45]
100974092  CD46; membrane cofactor protein isoform X4 [KO:K04007]
100983678  CD55; complement decay-accelerating factor isoform X4 [KO:K04006]
117975313  CFHR1; complement factor H-related protein 1 [KO:K23815]
117975341  CFHR3; LOW QUALITY PROTEIN: complement factor H-related protein 3 [KO:K23815]
100968959  complement factor H-related protein 4-like isoform X1 [KO:K23816]
100967817  CFHR5; complement factor H-related protein 5 [KO:K23817]
100991465  CD59; CD59 glycoprotein [KO:K04008]
100990129  CLU; clusterin isoform X1 [KO:K17252]
100980616  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
pps04611  Platelet activation
pps04662  B cell receptor signaling pathway
pps04750  Inflammatory mediator regulation of TRP channels
KO pathway
ko04610   

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