KEGG   PATHWAY: sbq04610
Entry
sbq04610                    Pathway                                
Name
Complement and coagulation cascades - Saimiri boliviensis boliviensis (Bolivian squirrel monkey)
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
sbq04610  Complement and coagulation cascades
sbq04610

Organism
Saimiri boliviensis boliviensis (Bolivian squirrel monkey) [GN:sbq]
Gene
101043113  F7; coagulation factor VII [KO:K01320] [EC:3.4.21.21]
101030131  F3; tissue factor [KO:K03901]
101051215  F12; coagulation factor XII [KO:K01328] [EC:3.4.21.38]
101048656  F11; coagulation factor XI isoform X1 [KO:K01323] [EC:3.4.21.27]
101046698  F9; coagulation factor IX [KO:K01321] [EC:3.4.21.22]
101044155  VWF; von Willebrand factor [KO:K03900]
101046407  F8; coagulation factor VIII [KO:K03899]
101048748  F10; coagulation factor X [KO:K01314] [EC:3.4.21.6]
101035896  F5; coagulation factor V [KO:K03902]
101049318  F2; prothrombin [KO:K01313] [EC:3.4.21.5]
101043642  FGA; LOW QUALITY PROTEIN: fibrinogen alpha chain [KO:K03903]
101043955  FGB; fibrinogen beta chain [KO:K03904]
101043315  FGG; fibrinogen gamma chain [KO:K03905]
101046928  F13A1; coagulation factor XIII A chain [KO:K03917] [EC:2.3.2.13]
101044451  coagulation factor XIII B chain isoform X1 [KO:K03906]
101048036  F2R; proteinase-activated receptor 1 [KO:K03914]
101030245  F2RL2; proteinase-activated receptor 3 isoform X1 [KO:K04235]
101029013  F2RL3; proteinase-activated receptor 4 [KO:K04236]
101041667  CPB2; carboxypeptidase B2 [KO:K01300] [EC:3.4.17.20]
101028591  THBD; thrombomodulin [KO:K03907]
101045065  PROCR; endothelial protein C receptor [KO:K06557]
101036326  PROC; vitamin K-dependent protein C isoform X3 [KO:K01344] [EC:3.4.21.69]
101043114  PROS1; vitamin K-dependent protein S [KO:K03908]
101045388  SERPINE1; plasminogen activator inhibitor 1 [KO:K03982]
101036715  SERPINB2; plasminogen activator inhibitor 2 [KO:K19821]
101038540  SERPINA5; plasma serine protease inhibitor [KO:K03913]
101037351  SERPINE2; glia-derived nexin [KO:K16643]
101046492  PLAT; tissue-type plasminogen activator [KO:K01343] [EC:3.4.21.68]
101045946  PLAU; urokinase-type plasminogen activator [KO:K01348] [EC:3.4.21.73]
101041224  PLAUR; urokinase plasminogen activator surface receptor [KO:K03985]
101028314  plasminogen [KO:K01315] [EC:3.4.21.7]
101048983  KLKB1; plasma kallikrein [KO:K01324] [EC:3.4.21.34]
101030410  KNG1; kininogen-1 isoform X1 [KO:K03898]
101044388  BDKRB1; B1 bradykinin receptor [KO:K03915]
101043867  BDKRB2; B2 bradykinin receptor [KO:K03916]
101041187  TFPI; tissue factor pathway inhibitor isoform X1 [KO:K03909]
101036735  SERPINA1; alpha-1-antitrypsin [KO:K03984]
101052447  SERPINC1; antithrombin-III [KO:K03911]
101050731  SERPIND1; heparin cofactor 2 [KO:K03912]
101047725  SERPING1; plasma protease C1 inhibitor [KO:K04001]
101042992  SERPINF2; alpha-2-antiplasmin [KO:K03983]
101030613  A2M; alpha-2-macroglobulin [KO:K03910]
101048882  C1QA; complement C1q subcomponent subunit A [KO:K03986]
101049399  C1QB; complement C1q subcomponent subunit B [KO:K03987]
101048354  C1QC; complement C1q subcomponent subunit C [KO:K03988]
101033061  C1R; complement C1r subcomponent isoform X2 [KO:K01330] [EC:3.4.21.41]
101034947  C1S; complement C1s subcomponent [KO:K01331] [EC:3.4.21.42]
101029843  mannose-binding protein A-like [KO:K03991]
101045108  MBL2; mannose-binding protein C [KO:K03991]
101033147  MASP1; mannan-binding lectin serine protease 1 isoform X1 [KO:K03992] [EC:3.4.21.-]
101047723  MASP2; mannan-binding lectin serine protease 2 isoform X1 [KO:K03993] [EC:3.4.21.104]
101045782  C4A; complement C4-A [KO:K03989]
104650316  C2; complement C2 [KO:K01332] [EC:3.4.21.43]
101044671  C3; complement C3 [KO:K03990]
101036079  CFB; complement factor B [KO:K01335] [EC:3.4.21.47]
101030841  CFD; complement factor D [KO:K01334] [EC:3.4.21.46]
101040214  C5; complement C5 [KO:K03994]
101028369  C6; complement component C6 [KO:K03995]
101029227  C7; complement component C7 [KO:K03996]
101046777  C8A; complement component C8 alpha chain [KO:K03997]
101047099  C8B; complement component C8 beta chain [KO:K03998]
101034811  C8G; complement component C8 gamma chain [KO:K03999]
101034195  C9; complement component C9 [KO:K04000]
101038526  C3AR1; C3a anaphylatoxin chemotactic receptor [KO:K04009]
101031130  VSIG4; V-set and immunoglobulin domain-containing protein 4 isoform X2 [KO:K19822]
104651759  CR1L; complement component receptor 1-like protein isoform X1 [KO:K04011]
101048758  CR1; complement receptor type 1 [KO:K04011]
101045483  CR2; complement receptor type 2 isoform X2 [KO:K04012]
101035688  ITGAM; integrin alpha-M isoform X1 [KO:K06461]
101053620  ITGB2; integrin beta-2 [KO:K06464]
101038763  ITGAX; integrin alpha-X [KO:K06462]
101034910  C5AR1; C5a anaphylatoxin chemotactic receptor 1 [KO:K04010]
101047590  C4b-binding protein alpha chain-like [KO:K04002]
101047898  zona pellucida sperm-binding protein 3 receptor-like [KO:K04002]
101048226  C4BPB; C4b-binding protein beta chain isoform X1 [KO:K04003]
101045793  CFI; complement factor I isoform X1 [KO:K01333] [EC:3.4.21.45]
101044777  complement factor H isoform X1 [KO:K04004]
101044625  CD46; membrane cofactor protein isoform X1 [KO:K04007]
101046326  CD55; complement decay-accelerating factor [KO:K04006]
101035265  complement factor H-related protein 1-like [KO:K23815]
101029073  CD59; CD59 glycoprotein [KO:K04008]
101033729  CLU; clusterin [KO:K17252]
101045891  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
sbq04611  Platelet activation
sbq04662  B cell receptor signaling pathway
sbq04750  Inflammatory mediator regulation of TRP channels
KO pathway
ko04610   
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