KEGG   PATHWAY: mun04610
Entry
mun04610                    Pathway                                
Name
Complement and coagulation cascades - Meriones unguiculatus (Mongolian gerbil)
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
mun04610  Complement and coagulation cascades
mun04610

Organism
Meriones unguiculatus (Mongolian gerbil) [GN:mun]
Gene
110559856  F3; tissue factor [KO:K03901]
110558005  F11; coagulation factor XI isoform X1 [KO:K01323] [EC:3.4.21.27]
110539834  F9; coagulation factor IX isoform X1 [KO:K01321] [EC:3.4.21.22]
110548579  Vwf; von Willebrand factor [KO:K03900]
110542245  F8; coagulation factor VIII isoform X1 [KO:K03899]
110559730  coagulation factor X-like [KO:K01314] [EC:3.4.21.6]
110549928  F5; coagulation factor V [KO:K03902]
110549882  F2; prothrombin isoform X1 [KO:K01313] [EC:3.4.21.5]
110547218  Fga; fibrinogen alpha chain [KO:K03903]
110547216  Fgb; fibrinogen beta chain [KO:K03904]
110547217  Fgg; fibrinogen gamma chain isoform X1 [KO:K03905]
110553494  F13a1; coagulation factor XIII A chain isoform X1 [KO:K03917] [EC:2.3.2.13]
110564825  F13b; coagulation factor XIII B chain isoform X1 [KO:K03906]
110551097  F2r; proteinase-activated receptor 1 [KO:K03914]
110551088  F2rl2; proteinase-activated receptor 3 [KO:K04235]
110563537  F2rl3; proteinase-activated receptor 4 [KO:K04236]
110547470  Cpb2; carboxypeptidase B2 [KO:K01300] [EC:3.4.17.20]
110555879  Thbd; thrombomodulin [KO:K03907]
110550568  Procr; endothelial protein C receptor isoform X1 [KO:K06557]
110553446  Proc; vitamin K-dependent protein C isoform X1 [KO:K01344] [EC:3.4.21.69]
110565896  Pros1; vitamin K-dependent protein S [KO:K03908]
110566254  Serpine1; plasminogen activator inhibitor 1 [KO:K03982]
110549516  Serpinb2; plasminogen activator inhibitor 2 [KO:K19821]
110539989  Serpina5; plasma serine protease inhibitor [KO:K03913]
110552878  Serpine2; glia-derived nexin isoform X2 [KO:K16643]
110556621  Plat; tissue-type plasminogen activator isoform X1 [KO:K01343] [EC:3.4.21.68]
110552013  Plau; urokinase-type plasminogen activator [KO:K01348] [EC:3.4.21.73]
110542721  Plaur; urokinase plasminogen activator surface receptor isoform X1 [KO:K03985]
110554863  Plg; plasminogen isoform X1 [KO:K01315] [EC:3.4.21.7]
110558008  Klkb1; plasma kallikrein isoform X1 [KO:K01324] [EC:3.4.21.34]
110548116  Kng1; kininogen-1 isoform X1 [KO:K03898]
110555926  Bdkrb1; B1 bradykinin receptor [KO:K03915]
110555920  Bdkrb2; B2 bradykinin receptor isoform X1 [KO:K03916]
110551966  Tfpi; tissue factor pathway inhibitor isoform X1 [KO:K03909]
110542650  alpha-1-antitrypsin isoform X1 [KO:K03984]
132655346  alpha-1-antiproteinase-like [KO:K03984]
110560732  Serpinc1; antithrombin-III [KO:K03911]
110559215  Serpind1; heparin cofactor 2 [KO:K03912]
110551975  Serping1; plasma protease C1 inhibitor [KO:K04001]
110546218  Serpinf2; alpha-2-antiplasmin isoform X1 [KO:K03983]
110546072  alpha-2-macroglobulin [KO:K03910]
110545386  C1qa; complement C1q subcomponent subunit A [KO:K03986]
110545384  C1qb; complement C1q subcomponent subunit B [KO:K03987]
110545387  C1qc; complement C1q subcomponent subunit C [KO:K03988]
110548593  C1r; complement C1r subcomponent isoform X1 [KO:K01330] [EC:3.4.21.41]
110548590  C1s; complement C1s subcomponent [KO:K01331] [EC:3.4.21.42]
110554959  Mbl2; mannose-binding protein C isoform X1 [KO:K03991]
110561390  mannose-binding protein A-like [KO:K03991]
110548132  Masp1; mannan-binding lectin serine protease 1 isoform X1 [KO:K03992] [EC:3.4.21.-]
110548141  Masp2; mannan-binding lectin serine protease 2 isoform X1 [KO:K03993] [EC:3.4.21.104]
110560118  complement C4 [KO:K03989]
110560138  C2; complement C2 [KO:K01332] [EC:3.4.21.43]
110545607  C3; complement C3 [KO:K03990]
110560137  Cfb; complement factor B [KO:K01335] [EC:3.4.21.47]
110551364  Cfd; complement factor D isoform X1 [KO:K01334] [EC:3.4.21.46]
110563762  C5; complement C5 isoform X1 [KO:K03994]
110557743  C6; complement component C6 [KO:K03995]
110566207  C7; complement component C7 isoform X1 [KO:K03996]
110556365  C8a; complement component C8 alpha chain isoform X1 [KO:K03997]
110556362  C8b; complement component C8 beta chain isoform X1 [KO:K03998]
110557018  C8g; complement component C8 gamma chain isoform X1 [KO:K03999]
110559186  C9; complement component C9 isoform X1 [KO:K04000]
110556294  C3ar1; C3a anaphylatoxin chemotactic receptor isoform X2 [KO:K04009]
110540033  Vsig4; LOW QUALITY PROTEIN: V-set and immunoglobulin domain-containing protein 4 [KO:K19822]
110564544  Itgam; integrin alpha-M [KO:K06461]
110545086  Itgb2; integrin beta-2 [KO:K06464]
110553725  Itgax; integrin alpha-X isoform X1 [KO:K06462]
110561320  C5ar1; C5a anaphylatoxin chemotactic receptor 1 [KO:K04010]
110550318  C4bpa; C4b-binding protein alpha chain isoform X1 [KO:K04002]
110550320  zona pellucida sperm-binding protein 3 receptor-like isoform X1 [KO:K04002]
110550313  C4bpb; C4b-binding protein beta chain [KO:K04003]
110553204  Cfi; complement factor I isoform X1 [KO:K01333] [EC:3.4.21.45]
110564827  complement factor H-like isoform X1 [KO:K04004]
110551849  membrane cofactor protein-like [KO:K04007]
110563675  Cd46; membrane cofactor protein isoform X1 [KO:K04007]
110550315  Cd55; complement decay-accelerating factor isoform X1 [KO:K04006]
110556840  Cd59; CD59 glycoprotein [KO:K04008]
110559204  Clu; clusterin [KO:K17252]
110550131  Vtn; vitronectin isoform X2 [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
mun04611  Platelet activation
mun04662  B cell receptor signaling pathway
mun04750  Inflammatory mediator regulation of TRP channels
KO pathway
ko04610   
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