KEGG   PATHWAY: mcal04610
Entry
mcal04610                   Pathway                                
Name
Complement and coagulation cascades - Mus caroli (Ryukyu mouse)
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
mcal04610  Complement and coagulation cascades
mcal04610

Organism
Mus caroli (Ryukyu mouse) [GN:mcal]
Gene
110300360  F7; LOW QUALITY PROTEIN: coagulation factor VII [KO:K01320] [EC:3.4.21.21]
110291216  F3; tissue factor [KO:K03901]
110308153  F12; coagulation factor XII isoform X1 [KO:K01328] [EC:3.4.21.38]
110300267  F11; coagulation factor XI isoform X1 [KO:K01323] [EC:3.4.21.27]
110286779  F9; coagulation factor IX [KO:K01321] [EC:3.4.21.22]
110295806  Vwf; von Willebrand factor [KO:K03900]
110286244  F8; coagulation factor VIII [KO:K03899]
110300095  F10; coagulation factor X [KO:K01314] [EC:3.4.21.6]
110301290  F5; coagulation factor V isoform X1 [KO:K03902]
110311945  F2; prothrombin [KO:K01313] [EC:3.4.21.5]
110291187  Fga; fibrinogen alpha chain [KO:K03903]
110291148  Fgb; fibrinogen beta chain [KO:K03904]
110291235  Fgg; fibrinogen gamma chain isoform X1 [KO:K03905]
110308340  F13a1; coagulation factor XIII A chain [KO:K03917] [EC:2.3.2.13]
110292378  F13b; coagulation factor XIII B chain isoform X1 [KO:K03906]
110308051  F2r; proteinase-activated receptor 1 [KO:K03914]
110308368  F2rl2; proteinase-activated receptor 3 [KO:K04235]
110300734  F2rl3; proteinase-activated receptor 4 [KO:K04236]
110309374  Cpb2; carboxypeptidase B2 [KO:K01300] [EC:3.4.17.20]
110289967  Thbd; thrombomodulin [KO:K03907]
110290154  Procr; endothelial protein C receptor isoform X1 [KO:K06557]
110284793  Proc; vitamin K-dependent protein C isoform X1 [KO:K01344] [EC:3.4.21.69]
110311444  Pros1; vitamin K-dependent protein S [KO:K03908]
110302177  Serpinb2; plasminogen activator inhibitor 2 [KO:K19821]
110306914  Serpina5; plasma serine protease inhibitor [KO:K03913]
110301753  Serpine2; glia-derived nexin [KO:K16643]
110300159  Plat; tissue-type plasminogen activator [KO:K01343] [EC:3.4.21.68]
110309656  Plau; urokinase-type plasminogen activator [KO:K01348] [EC:3.4.21.73]
110298984  Plaur; urokinase plasminogen activator surface receptor [KO:K03985]
110312566  Plg; plasminogen [KO:K01315] [EC:3.4.21.7]
110299702  Klkb1; plasma kallikrein [KO:K01324] [EC:3.4.21.34]
110311183  kininogen-1 isoform X1 [KO:K03898]
110307526  Bdkrb1; B1 bradykinin receptor [KO:K03915]
110307378  Bdkrb2; B2 bradykinin receptor [KO:K03916]
110288305  Tfpi; tissue factor pathway inhibitor isoform X1 [KO:K03909]
110307406  alpha-1-antitrypsin 1-3 [KO:K03984]
110306582  alpha-1-antitrypsin-like [KO:K03984]
110305104  Serpinc1; antithrombin-III [KO:K03911]
110311406  Serpind1; heparin cofactor 2 [KO:K03912]
110287173  Serping1; plasma protease C1 inhibitor [KO:K04001]
110305320  Serpinf2; alpha-2-antiplasmin [KO:K03983]
110293043  C1qa; complement C1q subcomponent subunit A [KO:K03986]
110292976  C1qb; complement C1q subcomponent subunit B [KO:K03987]
110293122  C1qc; complement C1q subcomponent subunit C [KO:K03988]
110309221  mannose-binding protein A [KO:K03991]
110285489  Mbl2; mannose-binding protein C [KO:K03991]
110311194  Masp1; mannan-binding lectin serine protease 1 isoform X1 [KO:K03992] [EC:3.4.21.-]
110292278  Masp2; mannan-binding lectin serine protease 2 isoform X1 [KO:K03993] [EC:3.4.21.104]
110283979  complement C4-B [KO:K03989]
110312387  C2; complement C2 [KO:K01332] [EC:3.4.21.43]
110312494  C3; complement C3 [KO:K03990]
110312388  Cfb; complement factor B isoform X1 [KO:K01335] [EC:3.4.21.47]
110303909  Cfd; complement factor D isoform X1 [KO:K01334] [EC:3.4.21.46]
110285518  C5; complement C5 [KO:K03994]
110310600  C6; complement component C6 [KO:K03995]
110310957  C7; complement component C7 [KO:K03996]
110292511  C8a; complement component C8 alpha chain isoform X1 [KO:K03997]
110292205  C8b; complement component C8 beta chain isoform X2 [KO:K03998]
110284736  C8g; complement component C8 gamma chain [KO:K03999]
110310523  C9; complement component C9 [KO:K04000]
110297056  C3ar1; C3a anaphylatoxin chemotactic receptor [KO:K04009]
110286958  Vsig4; V-set and immunoglobulin domain-containing protein 4 [KO:K19822]
110289966  Cr1l; LOW QUALITY PROTEIN: complement component receptor 1-like protein [KO:K04011]
110289952  Cr2; complement receptor type 2 isoform X2 [KO:K04012]
110298307  Itgam; integrin alpha-M isoform X2 [KO:K06461]
110311769  integrin beta-2-like protein [KO:K06464]
110303281  Itgb2; integrin beta-2 [KO:K06464]
110298506  integrin alpha-X isoform X1 [KO:K06462]
110299315  C5ar1; C5a anaphylatoxin chemotactic receptor 1 isoform X2 [KO:K04010]
110293221  zona pellucida sperm-binding protein 3 receptor [KO:K04002]
110291790  C4b-binding protein isoform X1 [KO:K04002]
110296041  C4bpb; LOW QUALITY PROTEIN: C4b-binding protein beta chain [KO:K04003]
110291924  Cfi; complement factor I [KO:K01333] [EC:3.4.21.45]
110288786  complement factor H [KO:K04004]
110299161  Cd46; membrane cofactor protein isoform X1 [KO:K04007]
110303044  Cd55; complement decay-accelerating factor [KO:K04006]
110289342  Cd59; CD59 glycoprotein [KO:K04008]
110309212  Clu; clusterin [KO:K17252]
110305821  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
mcal04611  Platelet activation
mcal04662  B cell receptor signaling pathway
mcal04750  Inflammatory mediator regulation of TRP channels
KO pathway
ko04610   
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