KEGG   PATHWAY: sara04610
Entry
sara04610                   Pathway                                
Name
Complement and coagulation cascades - Sorex araneus (European shrew)
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
sara04610  Complement and coagulation cascades
sara04610

Organism
Sorex araneus (European shrew) [GN:sara]
Gene
101537018  PROS1; vitamin K-dependent protein S [KO:K03908]
101537488  THBD; thrombomodulin [KO:K03907]
101538105  F12; coagulation factor XII [KO:K01328] [EC:3.4.21.38]
101538798  PROC; vitamin K-dependent protein C [KO:K01344] [EC:3.4.21.69]
101538899  [KO:K04004]
101538914  C8G; complement component C8 gamma chain [KO:K03999]
101539217  ITGB2; integrin beta-2 [KO:K06464]
101539316  SERPINF2; alpha-2-antiplasmin [KO:K03983]
101539723  BDKRB2; B2 bradykinin receptor [KO:K03916]
101539989  BDKRB1; B1 bradykinin receptor [KO:K03915]
101540190  MASP1; mannan-binding lectin serine protease 1 isoform X1 [KO:K03992] [EC:3.4.21.-]
101540741  VWF; von Willebrand factor [KO:K03900]
101540747  CFI; complement factor I [KO:K01333] [EC:3.4.21.45]
101540902  PLAT; tissue-type plasminogen activator [KO:K01343] [EC:3.4.21.68]
101540995  [KO:K03991]
101541015  F5; coagulation factor V [KO:K03902]
101541071  [KO:K03905]
101541147  KNG1; kininogen-1 [KO:K03898]
101541333  FGA; fibrinogen alpha chain [KO:K03903]
101541586  C1QB; complement C1q subcomponent subunit B [KO:K03987]
101541690  [KO:K03905]
101541698  CD59; CD59 glycoprotein [KO:K04008]
101541947  C7; complement component C7 [KO:K03996]
101541948  FGB; fibrinogen beta chain [KO:K03904]
101541986  PLAU; urokinase-type plasminogen activator [KO:K01348] [EC:3.4.21.73]
101542103  [KO:K01335] [EC:3.4.21.47]
101542832  F8; coagulation factor VIII [KO:K03899]
101543016  C9; LOW QUALITY PROTEIN: complement component C9 [KO:K04000]
101543720  SERPINA1; alpha-1-antitrypsin [KO:K03984]
101544230  F2R; proteinase-activated receptor 1 [KO:K03914]
101545594  CLU; clusterin [KO:K17252]
101545859  PROCR; endothelial protein C receptor [KO:K06557]
101546501  F2; prothrombin [KO:K01313] [EC:3.4.21.5]
101546603  F10; coagulation factor X [KO:K01314] [EC:3.4.21.6]
101546858  F7; coagulation factor VII [KO:K01320] [EC:3.4.21.21]
101546916  ITGAX; integrin alpha-X [KO:K06462]
101547063  C1S; complement C1s subcomponent [KO:K01331] [EC:3.4.21.42]
101547202  SERPINB2; plasminogen activator inhibitor 2 [KO:K19821]
101547327  C1R; complement C1r subcomponent [KO:K01330] [EC:3.4.21.41]
101548269  CFH; complement factor H [KO:K04004]
101548334  F3; tissue factor [KO:K03901]
101548448  SERPINE1; plasminogen activator inhibitor 1 [KO:K03982]
101548530  F13A1; coagulation factor XIII A chain [KO:K03917] [EC:2.3.2.13]
101549359  F9; coagulation factor IX isoform X1 [KO:K01321] [EC:3.4.21.22]
101549442  C1QA; complement C1q subcomponent subunit A [KO:K03986]
101549550  C8B; complement component C8 beta chain [KO:K03998]
101549707  C1QC; complement C1q subcomponent subunit C [KO:K03988]
101549815  C8A; complement component C8 alpha chain [KO:K03997]
101549854  A2M; alpha-2-macroglobulin [KO:K03910]
101550122  [KO:K03989]
101550576  CPB2; carboxypeptidase B2 [KO:K01300] [EC:3.4.17.20]
101550684  F13B; coagulation factor XIII B chain [KO:K03906]
101550718  C3; complement C3 [KO:K03990]
101550876  ITGAM; integrin alpha-M [KO:K06461]
101550987  [KO:K03990]
101551126  F11; coagulation factor XI [KO:K01323] [EC:3.4.21.27]
101551204  C6; complement component C6 [KO:K03995]
101551391  KLKB1; plasma kallikrein [KO:K01324] [EC:3.4.21.34]
101551835  C3AR1; C3a anaphylatoxin chemotactic receptor [KO:K04009]
101552888  MBL2; mannose-binding protein C [KO:K03991]
101553017  CR2; complement receptor type 2 [KO:K04012]
101553545  [KO:K04002]
101553713  CFD; complement factor D [KO:K01334] [EC:3.4.21.46]
101553809  C4BPB; C4b-binding protein beta chain [KO:K04003]
101553832  TFPI; tissue factor pathway inhibitor [KO:K03909]
101554126  MASP2; mannan-binding lectin serine protease 2 [KO:K03993] [EC:3.4.21.104]
101554648  VTN; vitronectin [KO:K06251]
101555169  PLAUR; urokinase plasminogen activator surface receptor [KO:K03985]
101555842  SERPINE2; glia-derived nexin [KO:K16643]
101556725  SERPINC1; antithrombin-III [KO:K03911]
101557429  C5AR1; C5a anaphylatoxin chemotactic receptor 1 [KO:K04010]
101557844  PLG; plasminogen [KO:K01315] [EC:3.4.21.7]
101558268  C5; complement C5 [KO:K03994]
101559345  F2RL3; proteinase-activated receptor 4 [KO:K04236]
105943174  F2RL2; proteinase-activated receptor 3 [KO:K04235]
105943273  CD55; complement decay-accelerating factor [KO:K04006]
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
sara04611  Platelet activation
sara04662  B cell receptor signaling pathway
sara04750  Inflammatory mediator regulation of TRP channels
KO pathway
ko04610   

DBGET integrated database retrieval system