KEGG   PATHWAY: eju04610
Entry
eju04610                    Pathway                                
Name
Complement and coagulation cascades - Eumetopias jubatus (Steller sea lion)
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
eju04610  Complement and coagulation cascades
eju04610

Organism
Eumetopias jubatus (Steller sea lion) [GN:eju]
Gene
114196651  F7; coagulation factor VII isoform X1 [KO:K01320] [EC:3.4.21.21]
114207837  F3; tissue factor isoform X1 [KO:K03901]
114208722  F12; coagulation factor XII isoform X1 [KO:K01328] [EC:3.4.21.38]
114206166  F11; coagulation factor XI isoform X1 [KO:K01323] [EC:3.4.21.27]
114224597  F9; coagulation factor IX isoform X1 [KO:K01321] [EC:3.4.21.22]
114222568  VWF; von Willebrand factor [KO:K03900]
114201915  F8; coagulation factor VIII isoform X1 [KO:K03899]
114196630  F10; coagulation factor X isoform X1 [KO:K01314] [EC:3.4.21.6]
114223957  F5; coagulation factor V isoform X1 [KO:K03902]
114215280  F2; prothrombin isoform X1 [KO:K01313] [EC:3.4.21.5]
114210153  FGA; fibrinogen alpha chain [KO:K03903]
114210152  FGB; fibrinogen beta chain isoform X1 [KO:K03904]
114210086  FGG; fibrinogen gamma chain isoform X1 [KO:K03905]
114223909  F13A1; coagulation factor XIII A chain [KO:K03917] [EC:2.3.2.13]
114222007  F13B; coagulation factor XIII B chain [KO:K03906]
114217620  F2R; proteinase-activated receptor 1 [KO:K03914]
114217559  F2RL2; proteinase-activated receptor 3 [KO:K04235]
114196466  F2RL3; proteinase-activated receptor 4 isoform X1 [KO:K04236]
114212061  CPB2; carboxypeptidase B2 isoform X1 [KO:K01300] [EC:3.4.17.20]
114224507  THBD; thrombomodulin [KO:K03907]
114220712  PROCR; endothelial protein C receptor [KO:K06557]
114207290  PROC; vitamin K-dependent protein C [KO:K01344] [EC:3.4.21.69]
114219374  PROS1; vitamin K-dependent protein S [KO:K03908]
114214452  SERPINE1; plasminogen activator inhibitor 1 [KO:K03982]
114208913  SERPINB2; plasminogen activator inhibitor 2 [KO:K19821]
114208182  SERPINA5; plasma serine protease inhibitor isoform X1 [KO:K03913]
114210781  SERPINE2; glia-derived nexin isoform X1 [KO:K16643]
114224827  PLAT; tissue-type plasminogen activator [KO:K01343] [EC:3.4.21.68]
114214232  PLAU; urokinase-type plasminogen activator [KO:K01348] [EC:3.4.21.73]
114197084  PLAUR; urokinase plasminogen activator surface receptor [KO:K03985]
114209283  PLG; plasminogen isoform X1 [KO:K01315] [EC:3.4.21.7]
114206167  KLKB1; plasma kallikrein isoform X1 [KO:K01324] [EC:3.4.21.34]
114207026  KNG1; kininogen-1 isoform X1 [KO:K03898]
114209011  BDKRB1; B1 bradykinin receptor [KO:K03915]
114210163  BDKRB2; B2 bradykinin receptor [KO:K03916]
114225315  TFPI; tissue factor pathway inhibitor [KO:K03909]
114205398  SERPINA1; alpha-1-antitrypsin isoform X1 [KO:K03984]
114223967  SERPINC1; antithrombin-III [KO:K03911]
114201949  SERPIND1; heparin cofactor 2 [KO:K03912]
114215316  SERPING1; plasma protease C1 inhibitor [KO:K04001]
114213926  SERPINF2; alpha-2-antiplasmin [KO:K03983]
114221675  alpha-2-macroglobulin-like [KO:K03910]
114199063  C1QC; complement C1q subcomponent subunit C [KO:K03988]
114222545  C1R; complement C1r subcomponent isoform X1 [KO:K01330] [EC:3.4.21.41]
114222555  C1S; complement C1s subcomponent [KO:K01331] [EC:3.4.21.42]
114214069  MBL2; LOW QUALITY PROTEIN: mannose-binding protein C [KO:K03991]
114207048  MASP1; mannan-binding lectin serine protease 1 [KO:K03992] [EC:3.4.21.-]
114213204  MASP2; mannan-binding lectin serine protease 2 isoform X1 [KO:K03993] [EC:3.4.21.104]
114222096  complement C4-B-like [KO:K03989]
114222260  C2; complement C2 [KO:K01332] [EC:3.4.21.43]
114226149  C3; complement C3 [KO:K03990]
114222131  CFB; complement factor B isoform X1 [KO:K01335] [EC:3.4.21.47]
114226342  CFD; complement factor D isoform X1 [KO:K01334] [EC:3.4.21.46]
114212820  C5; complement C5 [KO:K03994]
114204832  C6; complement component C6 [KO:K03995]
114204794  C7; complement component C7 isoform X1 [KO:K03996]
114212130  C8A; complement component C8 alpha chain [KO:K03997]
114212140  C8B; complement component C8 beta chain [KO:K03998]
114201774  C8G; complement component C8 gamma chain isoform X1 [KO:K03999]
114204828  C9; complement component C9 [KO:K04000]
114222571  C3AR1; C3a anaphylatoxin chemotactic receptor [KO:K04009]
114226462  VSIG4; V-set and immunoglobulin domain-containing protein 4 [KO:K19822]
114221931  CR2; complement receptor type 2 isoform X1 [KO:K04012]
114214337  ITGAM; integrin alpha-M isoform X1 [KO:K06461]
114226448  ITGB2; LOW QUALITY PROTEIN: integrin beta-2 [KO:K06464]
114214336  integrin alpha-X-like isoform X1 [KO:K06462]
114197037  C5AR1; C5a anaphylatoxin chemotactic receptor 1 [KO:K04010]
114221876  C4BPA; C4b-binding protein alpha chain isoform X1 [KO:K04002]
114221879  C4BPB; LOW QUALITY PROTEIN: C4b-binding protein beta chain [KO:K04003]
114222678  CFI; complement factor I [KO:K01333] [EC:3.4.21.45]
114202783  CFH; complement factor H [KO:K04004]
114221890  complement factor H-like [KO:K04004]
114221943  CD55; complement decay-accelerating factor isoform X1 [KO:K04006]
114210430  complement factor H-related protein 3-like [KO:K23815]
114222886  complement factor H-related protein 2-like [KO:K23815]
114222014  CFHR5; complement factor H-related protein 5 isoform X1 [KO:K23817]
114196928  CD59; CD59 glycoprotein [KO:K04008]
114217507  CLU; clusterin isoform X1 [KO:K17252]
114213730  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
eju04611  Platelet activation
eju04662  B cell receptor signaling pathway
eju04750  Inflammatory mediator regulation of TRP channels
KO pathway
ko04610   
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