KEGG   PATHWAY: mlx04610
Entry
mlx04610                    Pathway                                
Name
Complement and coagulation cascades - Mirounga leonina (Southern elephant seal)
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
mlx04610  Complement and coagulation cascades
mlx04610

Organism
Mirounga leonina (Southern elephant seal) [GN:mlx]
Gene
117998088  PLAUR; urokinase plasminogen activator surface receptor [KO:K03985]
117998197  C5AR1; C5a anaphylatoxin chemotactic receptor 1 [KO:K04010]
117999438  SERPINE1; plasminogen activator inhibitor 1 [KO:K03982]
117999556  [KO:K06462]
117999558  ITGAM; integrin alpha-M isoform X1 [KO:K06461]
117999817  SERPIND1; heparin cofactor 2 [KO:K03912]
118000407  KNG1; kininogen-1 isoform X1 [KO:K03898]
118001788  [KO:K01315] [EC:3.4.21.7]
118001906  [KO:K01315] [EC:3.4.21.7]
118002013  BDKRB2; B2 bradykinin receptor isoform X1 [KO:K03916]
118002184  SERPINA5; plasma serine protease inhibitor [KO:K03913]
118002481  BDKRB1; LOW QUALITY PROTEIN: B1 bradykinin receptor [KO:K03915]
118004523  SERPINB2; plasminogen activator inhibitor 2 [KO:K19821]
118004908  C3; complement C3 [KO:K03990]
118005033  CFD; complement factor D [KO:K01334] [EC:3.4.21.46]
118005387  F2RL3; proteinase-activated receptor 4 [KO:K04236]
118006335  [KO:K04000]
118006357  [KO:K03995]
118006423  [KO:K03996]
118006469  [KO:K04000]
118006559  [KO:K03996]
118006570  [KO:K03995]
118006928  [KO:K03992] [EC:3.4.21.-]
118009111  F10; coagulation factor X isoform X1 [KO:K01314] [EC:3.4.21.6]
118009112  F7; coagulation factor VII isoform X1 [KO:K01320] [EC:3.4.21.21]
118009180  CPB2; carboxypeptidase B2 [KO:K01300] [EC:3.4.17.20]
118009922  PLAT; tissue-type plasminogen activator [KO:K01343] [EC:3.4.21.68]
118010046  KLKB1; plasma kallikrein [KO:K01324] [EC:3.4.21.34]
118010047  F11; coagulation factor XI isoform X1 [KO:K01323] [EC:3.4.21.27]
118011182  THBD; thrombomodulin [KO:K03907]
118011416  PROCR; endothelial protein C receptor [KO:K06557]
118011743  F9; coagulation factor IX isoform X1 [KO:K01321] [EC:3.4.21.22]
118011995  SERPINE2; glia-derived nexin isoform X1 [KO:K16643]
118012233  TFPI; tissue factor pathway inhibitor isoform X1 [KO:K03909]
118012569  PROC; vitamin K-dependent protein C isoform X1 [KO:K01344] [EC:3.4.21.69]
118013693  F13B; coagulation factor XIII B chain [KO:K03906]
118013698  C4BPA; C4b-binding protein alpha chain isoform X1 [KO:K04002]
118013716  [KO:K23815]
118014121  [KO:K04004]
118014247  CFHR5; complement factor H-related protein 5 [KO:K23817]
118014258  C4BPB; C4b-binding protein beta chain [KO:K04003]
118014259  CD55; complement decay-accelerating factor isoform X1 [KO:K04006]
118014260  CR2; complement receptor type 2 isoform X1 [KO:K04012]
118014262  CR1L; complement component receptor 1-like protein [KO:K04011]
118014263  [KO:K04007]
118014264  [KO:K04007]
118014379  F8; coagulation factor VIII isoform X1 [KO:K03899]
118014454  SERPINC1; antithrombin-III [KO:K03911]
118014502  F5; coagulation factor V [KO:K03902]
118014694  [KO:K03984]
118015038  VSIG4; V-set and immunoglobulin domain-containing protein 4 isoform X1 [KO:K19822]
118015568  F2; prothrombin [KO:K01313] [EC:3.4.21.5]
118015747  CD59; CD59 glycoprotein [KO:K04008]
118015865  SERPING1; plasma protease C1 inhibitor [KO:K04001]
118016913  F2RL2; proteinase-activated receptor 3 [KO:K04235]
118016916  F2R; proteinase-activated receptor 1 isoform X1 [KO:K03914]
118017315  F12; coagulation factor XII isoform X1 [KO:K01328] [EC:3.4.21.38]
118017350  PROS1; vitamin K-dependent protein S [KO:K03908]
118017502  C8G; complement component C8 gamma chain isoform X1 [KO:K03999]
118017747  C5; complement C5 [KO:K03994]
118019120  F3; tissue factor [KO:K03901]
118019364  C8B; complement component C8 beta chain [KO:K03998]
118019908  C1QB; complement C1q subcomponent subunit B [KO:K03987]
118019909  C1QC; complement C1q subcomponent subunit C [KO:K03988]
118019910  C1QA; complement C1q subcomponent subunit A [KO:K03986]
118020013  MASP2; mannan-binding lectin serine protease 2 [KO:K03993] [EC:3.4.21.104]
118020219  C8A; complement component C8 alpha chain [KO:K03997]
118021790  PLAU; urokinase-type plasminogen activator [KO:K01348] [EC:3.4.21.73]
118022114  CLU; clusterin [KO:K17252]
118022221  FGB; fibrinogen beta chain isoform X1 [KO:K03904]
118022248  MBL2; mannose-binding protein C [KO:K03991]
118022438  FGG; fibrinogen gamma chain isoform X1 [KO:K03905]
118022440  FGA; fibrinogen alpha chain [KO:K03903]
118025214  SERPINF2; alpha-2-antiplasmin [KO:K03983]
118025294  VTN; vitronectin [KO:K06251]
118025690  [KO:K03910]
118025701  C3AR1; C3a anaphylatoxin chemotactic receptor [KO:K04009]
118025712  C1R; complement C1r subcomponent [KO:K01330] [EC:3.4.21.41]
118025713  C1S; complement C1s subcomponent [KO:K01331] [EC:3.4.21.42]
118025754  VWF; von Willebrand factor [KO:K03900]
118026948  ITGB2; integrin beta-2 isoform X1 [KO:K06464]
118027090  CFI; complement factor I [KO:K01333] [EC:3.4.21.45]
118027309  [KO:K03989]
118027314  CFB; complement factor B [KO:K01335] [EC:3.4.21.47]
118027318  C2; complement C2 [KO:K01332] [EC:3.4.21.43]
118027422  F13A1; coagulation factor XIII A chain [KO:K03917] [EC:2.3.2.13]
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
mlx04611  Platelet activation
mlx04662  B cell receptor signaling pathway
mlx04750  Inflammatory mediator regulation of TRP channels
KO pathway
ko04610   

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