KEGG   PATHWAY: elk04610
Entry
elk04610                    Pathway                                
Name
Complement and coagulation cascades - Enhydra lutris kenyoni (northern sea otter)
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
elk04610  Complement and coagulation cascades
elk04610

Organism
Enhydra lutris kenyoni (northern sea otter) [GN:elk]
Gene
111147145  coagulation factor VII isoform X1 [KO:K01320] [EC:3.4.21.21]
111143268  tissue factor isoform X1 [KO:K03901]
111141160  coagulation factor XII isoform X1 [KO:K01328] [EC:3.4.21.38]
111144552  coagulation factor XI isoform X1 [KO:K01323] [EC:3.4.21.27]
111158974  coagulation factor IX isoform X1 [KO:K01321] [EC:3.4.21.22]
111159823  von Willebrand factor isoform X1 [KO:K03900]
111139148  LOW QUALITY PROTEIN: coagulation factor VIII [KO:K03899]
111146998  coagulation factor X isoform X1 [KO:K01314] [EC:3.4.21.6]
111150744  coagulation factor V [KO:K03902]
111149799  prothrombin isoform X1 [KO:K01313] [EC:3.4.21.5]
111154842  fibrinogen alpha chain [KO:K03903]
111157895  fibrinogen beta chain [KO:K03904]
111153669  fibrinogen gamma chain isoform X1 [KO:K03905]
111156704  coagulation factor XIII A chain [KO:K03917] [EC:2.3.2.13]
111151135  coagulation factor XIII B chain [KO:K03906]
111148319  proteinase-activated receptor 1 [KO:K03914]
111148216  proteinase-activated receptor 3 [KO:K04235]
111144372  LOW QUALITY PROTEIN: proteinase-activated receptor 4 [KO:K04236]
111147094  carboxypeptidase B2 [KO:K01300] [EC:3.4.17.20]
111141662  thrombomodulin [KO:K03907]
111141722  endothelial protein C receptor isoform X1 [KO:K06557]
111160850  vitamin K-dependent protein C isoform X1 [KO:K01344] [EC:3.4.21.69]
111148875  vitamin K-dependent protein S [KO:K03908]
111145849  plasminogen activator inhibitor 1 [KO:K03982]
111154270  plasminogen activator inhibitor 2 [KO:K19821]
111148596  plasma serine protease inhibitor isoform X1 [KO:K03913]
111142041  glia-derived nexin [KO:K16643]
111144489  tissue-type plasminogen activator [KO:K01343] [EC:3.4.21.68]
111144976  urokinase-type plasminogen activator isoform X1 [KO:K01348] [EC:3.4.21.73]
111139531  urokinase plasminogen activator surface receptor isoform X1 [KO:K03985]
111158701  plasminogen [KO:K01315] [EC:3.4.21.7]
111144530  plasma kallikrein [KO:K01324] [EC:3.4.21.34]
111148821  kininogen-1 isoform X1 [KO:K03898]
111148793  B1 bradykinin receptor [KO:K03915]
111148709  B2 bradykinin receptor [KO:K03916]
111145643  tissue factor pathway inhibitor [KO:K03909]
111148485  alpha-1-antitrypsin [KO:K03984]
111150927  antithrombin-III [KO:K03911]
111142397  heparin cofactor 2 [KO:K03912]
111149560  plasma protease C1 inhibitor [KO:K04001]
111151239  alpha-2-antiplasmin isoform X1 [KO:K03983]
111142180  alpha-2-macroglobulin [KO:K03910]
111159719  complement C1r subcomponent [KO:K01330] [EC:3.4.21.41]
111159747  complement C1s subcomponent [KO:K01331] [EC:3.4.21.42]
111145397  LOW QUALITY PROTEIN: mannose-binding protein C [KO:K03991]
111147948  mannan-binding lectin serine protease 1 isoform X1 [KO:K03992] [EC:3.4.21.-]
111154837  mannan-binding lectin serine protease 2-like [KO:K03993] [EC:3.4.21.104]
111154710  mannan-binding lectin serine protease 2 [KO:K03993] [EC:3.4.21.104]
111152244  complement C4-A-like isoform X1 [KO:K03989]
111152312  complement C2 isoform X1 [KO:K01332] [EC:3.4.21.43]
111161841  complement C3 [KO:K03990]
111152198  complement factor B [KO:K01335] [EC:3.4.21.47]
111161686  complement factor D-like [KO:K01334] [EC:3.4.21.46]
111145939  complement C5 [KO:K03994]
111141090  complement component C6 [KO:K03995]
111140845  complement component C7 isoform X1 [KO:K03996]
111143421  LOW QUALITY PROTEIN: complement component C8 alpha chain [KO:K03997]
111143455  complement component C8 beta chain [KO:K03998]
111146056  complement component C8 gamma chain [KO:K03999]
111141260  complement component C9 [KO:K04000]
111159830  LOW QUALITY PROTEIN: C3a anaphylatoxin chemotactic receptor [KO:K04009]
111139325  V-set and immunoglobulin domain-containing protein 4 isoform X1 [KO:K19822]
111150949  complement receptor type 1-like [KO:K04011]
111162350  complement receptor type 1-like isoform X1 [KO:K04011]
111151001  complement receptor type 2 isoform X1 [KO:K04012]
111162313  integrin alpha-M [KO:K06461]
111155758  integrin beta-2 isoform X1 [KO:K06464]
111162188  integrin alpha-X isoform X1 [KO:K06462]
111160717  C5a anaphylatoxin chemotactic receptor 1 isoform X1 [KO:K04010]
111151046  C4b-binding protein alpha chain [KO:K04002]
111151098  C4b-binding protein beta chain [KO:K04003]
111155213  complement factor I [KO:K01333] [EC:3.4.21.45]
111150965  complement factor H-like [KO:K04004]
111162359  membrane cofactor protein-like isoform X1 [KO:K04007]
111162364  membrane cofactor protein-like [KO:K04007]
111151102  complement decay-accelerating factor isoform X1 [KO:K04006]
111150830  complement factor H-related protein 2 [KO:K23815]
111151085  complement factor H-related protein 5 isoform X1 [KO:K23817]
111149804  CD59 glycoprotein [KO:K04008]
111153483  clusterin [KO:K17252]
111151505  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
elk04611  Platelet activation
elk04662  B cell receptor signaling pathway
elk04750  Inflammatory mediator regulation of TRP channels
KO pathway
ko04610   
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