KEGG   PATHWAY: setr04610
Entry
setr04610                   Pathway                                
Name
Complement and coagulation cascades - Suncus etruscus (white-toothed pygmy 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
setr04610  Complement and coagulation cascades
setr04610

Organism
Suncus etruscus (white-toothed pygmy shrew) [GN:setr]
Gene
125995016  [KO:K03991]
125995017  [KO:K03991]
125996003  F13A1; LOW QUALITY PROTEIN: coagulation factor XIII A chain [KO:K03917] [EC:2.3.2.13]
125996183  C2; complement C2 [KO:K01332] [EC:3.4.21.43]
125996204  CFB; complement factor B [KO:K01335] [EC:3.4.21.47]
125996217  [KO:K03989]
125996393  PLG; plasminogen [KO:K01315] [EC:3.4.21.7]
125997340  F3; tissue factor [KO:K03901]
125998045  F2R; proteinase-activated receptor 1 [KO:K03914]
125998616  F8; coagulation factor VIII [KO:K03899]
125999442  F9; coagulation factor IX [KO:K01321] [EC:3.4.21.22]
125999858  C6; complement component C6 [KO:K03995]
125999901  C7; complement component C7 [KO:K03996]
126000419  C9; complement component C9 [KO:K04000]
126000978  SERPINE2; glia-derived nexin [KO:K16643]
126001008  F2RL2; LOW QUALITY PROTEIN: proteinase-activated receptor 3 [KO:K04235]
126003427  FGG; fibrinogen gamma chain [KO:K03905]
126003442  FGA; fibrinogen alpha chain [KO:K03903]
126003443  FGB; fibrinogen beta chain [KO:K03904]
126003498  CFH; complement factor H [KO:K04004]
126003501  F13B; coagulation factor XIII B chain [KO:K03906]
126003502  [KO:K23815]
126003579  [KO:K03984]
126003583  BDKRB2; B2 bradykinin receptor [KO:K03916]
126003591  BDKRB1; B1 bradykinin receptor [KO:K03915]
126004279  [KO:K03984]
126004297  C4BPB; C4b-binding protein beta chain [KO:K04003]
126004298  [KO:K04002]
126004299  [KO:K04006]
126004300  CR2; complement receptor type 2 [KO:K04012]
126004326  [KO:K04004]
126004327  [KO:K04004]
126004330  [KO:K23815]
126006425  PLAT; tissue-type plasminogen activator [KO:K01343] [EC:3.4.21.68]
126006454  VTN; vitronectin [KO:K06251]
126006501  CLU; clusterin isoform X1 [KO:K17252]
126006504  VWF; LOW QUALITY PROTEIN: von Willebrand factor [KO:K03900]
126006532  C1R; complement C1r subcomponent [KO:K01330] [EC:3.4.21.41]
126006556  C3AR1; C3a anaphylatoxin chemotactic receptor [KO:K04009]
126006663  C1S; LOW QUALITY PROTEIN: complement C1s subcomponent [KO:K01331] [EC:3.4.21.42]
126006893  KLKB1; plasma kallikrein [KO:K01324] [EC:3.4.21.34]
126006894  F11; coagulation factor XI [KO:K01323] [EC:3.4.21.27]
126008811  [KO:K03994]
126008854  PROC; vitamin K-dependent protein C [KO:K01344] [EC:3.4.21.69]
126009014  TFPI; tissue factor pathway inhibitor [KO:K03909]
126009297  C8G; complement component C8 gamma chain [KO:K03999]
126010982  C8B; complement component C8 beta chain [KO:K03998]
126010984  C8A; complement component C8 alpha chain [KO:K03997]
126011266  C1QB; complement C1q subcomponent subunit B [KO:K03987]
126011270  C1QA; complement C1q subcomponent subunit A [KO:K03986]
126011271  C1QC; complement C1q subcomponent subunit C [KO:K03988]
126011486  THBD; thrombomodulin [KO:K03907]
126011509  MASP2; mannan-binding lectin serine protease 2 [KO:K03993] [EC:3.4.21.104]
126011862  KNG1; LOW QUALITY PROTEIN: kininogen-1 [KO:K03898]
126011932  MASP1; mannan-binding lectin serine protease 1 isoform X1 [KO:K03992] [EC:3.4.21.-]
126012018  F12; coagulation factor XII [KO:K01328] [EC:3.4.21.38]
126013346  F5; coagulation factor V [KO:K03902]
126013422  SERPINC1; antithrombin-III [KO:K03911]
126015567  F10; coagulation factor X [KO:K01314] [EC:3.4.21.6]
126015568  F7; coagulation factor VII [KO:K01320] [EC:3.4.21.21]
126016325  CPB2; LOW QUALITY PROTEIN: carboxypeptidase B2 [KO:K01300] [EC:3.4.17.20]
126017428  PROCR; endothelial protein C receptor isoform X1 [KO:K06557]
126017623  SERPING1; plasma protease C1 inhibitor [KO:K04001]
126017921  F2; prothrombin [KO:K01313] [EC:3.4.21.5]
126018013  CD59; CD59 glycoprotein [KO:K04008]
126020588  SERPINB2; plasminogen activator inhibitor 2 [KO:K19821]
126020817  SERPINF2; alpha-2-antiplasmin isoform X1 [KO:K03983]
126022062  [KO:K03910]
126025916  ITGB2; integrin beta-2 [KO:K06464]
126026017  PROS1; vitamin K-dependent protein S [KO:K03908]
126027513  [KO:K04010]
126027514  [KO:K04010]
126027516  [KO:K04010]
126027527  [KO:K04010]
126027528  [KO:K04010]
126027566  CFI; complement factor I [KO:K01333] [EC:3.4.21.45]
126028251  PLAUR; urokinase plasminogen activator surface receptor [KO:K03985]
126028584  [KO:K04010]
126028585  [KO:K04010]
126028586  [KO:K04010]
126028588  [KO:K04010]
126028589  [KO:K04010]
126028590  [KO:K04010]
126028591  [KO:K04010]
126028592  [KO:K04010]
126028593  [KO:K04010]
126029597  SERPINE1; plasminogen activator inhibitor 1 isoform X1 [KO:K03982]
126030216  F2RL3; proteinase-activated receptor 4 [KO:K04236]
126030817  CFD; complement factor D [KO:K01334] [EC:3.4.21.46]
126030989  ITGAM; integrin alpha-M [KO:K06461]
126031002  PLAU; urokinase-type plasminogen activator [KO:K01348] [EC:3.4.21.73]
126031197  C3; complement C3 [KO:K03990]
126033682  SERPIND1; heparin cofactor 2 [KO:K03912]
126033725  [KO:K03991]
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
setr04611  Platelet activation
setr04662  B cell receptor signaling pathway
setr04750  Inflammatory mediator regulation of TRP channels
KO pathway
ko04610   

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