KEGG   PATHWAY: pcoq04610
Entry
pcoq04610                   Pathway                                
Name
Complement and coagulation cascades - Propithecus coquereli (Coquerel's sifaka)
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
pcoq04610  Complement and coagulation cascades
pcoq04610

Organism
Propithecus coquereli (Coquerel's sifaka) [GN:pcoq]
Gene
105825139  F7; coagulation factor VII [KO:K01320] [EC:3.4.21.21]
105823853  F3; tissue factor isoform X1 [KO:K03901]
105823511  F12; coagulation factor XII [KO:K01328] [EC:3.4.21.38]
105810113  F11; coagulation factor XI [KO:K01323] [EC:3.4.21.27]
105827247  F9; coagulation factor IX [KO:K01321] [EC:3.4.21.22]
105805087  VWF; von Willebrand factor [KO:K03900]
105824216  F8; coagulation factor VIII [KO:K03899]
105825137  coagulation factor X-like [KO:K01314] [EC:3.4.21.6]
105821084  F5; coagulation factor V [KO:K03902]
105811083  F2; prothrombin isoform X1 [KO:K01313] [EC:3.4.21.5]
105811122  FGA; fibrinogen alpha chain isoform X1 [KO:K03903]
105811121  FGB; fibrinogen beta chain [KO:K03904]
105811120  FGG; fibrinogen gamma chain isoform X1 [KO:K03905]
105812523  F13A1; coagulation factor XIII A chain [KO:K03917] [EC:2.3.2.13]
105815303  F13B; coagulation factor XIII B chain isoform X1 [KO:K03906]
105826501  F2R; proteinase-activated receptor 1 [KO:K03914]
105826610  F2RL2; proteinase-activated receptor 3 isoform X1 [KO:K04235]
105826285  F2RL3; proteinase-activated receptor 4 [KO:K04236]
105813572  CPB2; carboxypeptidase B2 [KO:K01300] [EC:3.4.17.20]
105817449  THBD; thrombomodulin [KO:K03907]
105827552  PROCR; endothelial protein C receptor isoform X1 [KO:K06557]
105814846  PROC; vitamin K-dependent protein C isoform X1 [KO:K01344] [EC:3.4.21.69]
105810359  PROS1; vitamin K-dependent protein S [KO:K03908]
105826028  SERPINE1; plasminogen activator inhibitor 1 [KO:K03982]
105811151  SERPINB2; plasminogen activator inhibitor 2 [KO:K19821]
105819045  SERPINA5; plasma serine protease inhibitor [KO:K03913]
105808803  SERPINE2; glia-derived nexin [KO:K16643]
105819695  PLAT; tissue-type plasminogen activator [KO:K01343] [EC:3.4.21.68]
105825510  PLAU; urokinase-type plasminogen activator [KO:K01348] [EC:3.4.21.73]
105817589  PLAUR; urokinase plasminogen activator surface receptor isoform X1 [KO:K03985]
105812401  PLG; LOW QUALITY PROTEIN: plasminogen [KO:K01315] [EC:3.4.21.7]
105810082  KLKB1; plasma kallikrein [KO:K01324] [EC:3.4.21.34]
105807671  KNG1; kininogen-1 isoform X1 [KO:K03898]
105814965  BDKRB1; B1 bradykinin receptor [KO:K03915]
105814963  BDKRB2; B2 bradykinin receptor [KO:K03916]
105825750  TFPI; tissue factor pathway inhibitor [KO:K03909]
105819056  alpha-1-antitrypsin-like [KO:K03984]
105821137  SERPINC1; antithrombin-III [KO:K03911]
105810192  SERPIND1; heparin cofactor 2 isoform X1 [KO:K03912]
105810048  SERPING1; plasma protease C1 inhibitor [KO:K04001]
105817352  SERPINF2; alpha-2-antiplasmin [KO:K03983]
105805007  alpha-2-macroglobulin-like [KO:K03910]
105815702  C1QA; complement C1q subcomponent subunit A [KO:K03986]
105815704  C1QB; complement C1q subcomponent subunit B [KO:K03987]
105815703  C1QC; complement C1q subcomponent subunit C [KO:K03988]
105805061  C1R; complement C1r subcomponent isoform X1 [KO:K01330] [EC:3.4.21.41]
105805031  C1S; complement C1s subcomponent [KO:K01331] [EC:3.4.21.42]
105825519  mannose-binding protein A-like isoform X1 [KO:K03991]
105822637  MBL2; mannose-binding protein C [KO:K03991]
105807740  MASP1; mannan-binding lectin serine protease 1 isoform X1 [KO:K03992] [EC:3.4.21.-]
105824943  MASP2; mannan-binding lectin serine protease 2 isoform X1 [KO:K03993] [EC:3.4.21.104]
105817862  C4A; complement C4-A [KO:K03989]
105817869  C2; complement C2 isoform X1 [KO:K01332] [EC:3.4.21.43]
105811847  C3; LOW QUALITY PROTEIN: complement C3 [KO:K03990]
105817868  CFB; complement factor B [KO:K01335] [EC:3.4.21.47]
105811278  CFD; complement factor D [KO:K01334] [EC:3.4.21.46]
105826411  C5; complement C5 [KO:K03994]
105814561  C6; complement component C6 [KO:K03995]
105814548  C7; complement component C7 [KO:K03996]
105806386  C8A; complement component C8 alpha chain [KO:K03997]
105806385  C8B; complement component C8 beta chain [KO:K03998]
105818693  C8G; complement component C8 gamma chain isoform X1 [KO:K03999]
105814532  C9; complement component C9 [KO:K04000]
105805071  C3AR1; C3a anaphylatoxin chemotactic receptor [KO:K04009]
105809831  VSIG4; V-set and immunoglobulin domain-containing protein 4 isoform X1 [KO:K19822]
105805845  CR1; complement receptor type 1 [KO:K04011]
105805844  CR2; complement receptor type 2 [KO:K04012]
105823085  integrin alpha-M-like [KO:K06461]
105817083  ITGB2; LOW QUALITY PROTEIN: integrin beta-2 [KO:K06464]
105817587  C5AR1; C5a anaphylatoxin chemotactic receptor 1 [KO:K04010]
105805874  C4BPA; C4b-binding protein alpha chain [KO:K04002]
105805843  C4BPB; C4b-binding protein beta chain [KO:K04003]
105818975  CFI; complement factor I [KO:K01333] [EC:3.4.21.45]
105815321  complement factor H-like isoform X1 [KO:K04004]
105805868  CD46; membrane cofactor protein isoform X1 [KO:K04007]
105805877  CD55; complement decay-accelerating factor isoform X1 [KO:K04006]
105814575  CD59; CD59 glycoprotein [KO:K04008]
105825253  CLU; clusterin isoform X1 [KO:K17252]
105813545  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
pcoq04611  Platelet activation
pcoq04662  B cell receptor signaling pathway
pcoq04750  Inflammatory mediator regulation of TRP channels
KO pathway
ko04610   
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