KEGG   PATHWAY: mpah04610
Entry
mpah04610                   Pathway                                
Name
Complement and coagulation cascades - Mus pahari (shrew 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
mpah04610  Complement and coagulation cascades
mpah04610

Organism
Mus pahari (shrew mouse) [GN:mpah]
Gene
110313068  Serpine1; plasminogen activator inhibitor 1 [KO:K03982]
110313611  F8; coagulation factor VIII isoform X1 [KO:K03899]
110314135  F9; coagulation factor IX [KO:K01321] [EC:3.4.21.22]
110314138  Vsig4; V-set and immunoglobulin domain-containing protein 4 [KO:K19822]
110314998  [KO:K01331] [EC:3.4.21.42]
110314999  [KO:K01330] [EC:3.4.21.41]
110315756  [KO:K23815]
110316081  A2m; alpha-2-macroglobulin [KO:K03910]
110316106  Vwf; von Willebrand factor [KO:K03900]
110316472  C1r; complement C1r subcomponent [KO:K01330] [EC:3.4.21.41]
110316909  C1s; complement C1s subcomponent [KO:K01331] [EC:3.4.21.42]
110317061  C3ar1; C3a anaphylatoxin chemotactic receptor [KO:K04009]
110317539  C5; complement C5 [KO:K03994]
110318150  C8g; complement component C8 gamma chain [KO:K03999]
110318522  Thbd; thrombomodulin [KO:K03907]
110318833  Serping1; plasma protease C1 inhibitor [KO:K04001]
110318900  Tfpi; tissue factor pathway inhibitor isoform X1 [KO:K03909]
110319115  F2; prothrombin isoform X1 [KO:K01313] [EC:3.4.21.5]
110319235  Procr; endothelial protein C receptor [KO:K06557]
110319661  [KO:K04008]
110319701  Cd59; CD59 glycoprotein [KO:K04008]
110320545  F3; tissue factor [KO:K03901]
110320605  Fgg; fibrinogen gamma chain isoform X1 [KO:K03905]
110320634  Cfi; complement factor I isoform X1 [KO:K01333] [EC:3.4.21.45]
110320684  Fgb; fibrinogen beta chain isoform X1 [KO:K03904]
110321050  Fga; fibrinogen alpha chain isoform X1 [KO:K03903]
110321620  Cr2; complement receptor type 2 [KO:K04012]
110321621  Cr1l; complement component receptor 1-like protein isoform X1 [KO:K04011]
110321775  Serpine2; glia-derived nexin isoform X1 [KO:K16643]
110322001  C4bpb; LOW QUALITY PROTEIN: C4b-binding protein beta chain [KO:K04003]
110322014  Cfh; complement factor H [KO:K04004]
110322103  C4bpa; C4b-binding protein alpha chain [KO:K04002]
110322113  F5; coagulation factor V [KO:K03902]
110322166  Cd55; complement decay-accelerating factor isoform X1 [KO:K04006]
110322312  [KO:K03906]
110322354  Cd46; membrane cofactor protein [KO:K04007]
110322355  Serpinb2; plasminogen activator inhibitor 2 [KO:K19821]
110322401  Serpinc1; antithrombin-III [KO:K03911]
110323447  C8b; complement component C8 beta chain isoform X1 [KO:K03998]
110323473  Masp2; mannan-binding lectin serine protease 2 isoform X1 [KO:K03993] [EC:3.4.21.104]
110323604  C1qb; complement C1q subcomponent subunit B [KO:K03987]
110323611  C1qc; complement C1q subcomponent subunit C [KO:K03988]
110323615  C8a; complement component C8 alpha chain [KO:K03997]
110323698  C1qa; complement C1q subcomponent subunit A [KO:K03986]
110324428  [KO:K03984]
110324489  [KO:K03984]
110324828  Serpina5; plasma serine protease inhibitor [KO:K03913]
110324855  Bdkrb2; B2 bradykinin receptor isoform X1 [KO:K03916]
110325005  Bdkrb1; B1 bradykinin receptor [KO:K03915]
110325498  Clu; clusterin [KO:K17252]
110325641  Plau; urokinase-type plasminogen activator [KO:K01348] [EC:3.4.21.73]
110325644  Cpb2; carboxypeptidase B2 [KO:K01300] [EC:3.4.17.20]
110325947  [KO:K03991]
110326926  Itgb2; integrin beta-2 [KO:K06464]
110326960  Cfd; complement factor D isoform X1 [KO:K01334] [EC:3.4.21.46]
110327271  Itgax; integrin alpha-X [KO:K06462]
110327528  Itgam; integrin alpha-M isoform X1 [KO:K06461]
110328865  F2rl2; proteinase-activated receptor 3 [KO:K04235]
110328891  C7; complement component C7 [KO:K03996]
110328985  F2r; proteinase-activated receptor 1 [KO:K03914]
110329162  C9; complement component C9 [KO:K04000]
110329165  C6; complement component C6 [KO:K03995]
110329478  Serpind1; heparin cofactor 2 [KO:K03912]
110329505  Masp1; mannan-binding lectin serine protease 1 [KO:K03992] [EC:3.4.21.-]
110329814  Pros1; vitamin K-dependent protein S [KO:K03908]
110329894  Kng1; kininogen-1 [KO:K03898]
110330025  [KO:K03898]
110330040  [KO:K06464]
110332069  Vtn; vitronectin [KO:K06251]
110332409  Serpinf2; alpha-2-antiplasmin isoform X1 [KO:K03983]
110333316  Proc; vitamin K-dependent protein C isoform X1 [KO:K01344] [EC:3.4.21.69]
110333906  F13a1; coagulation factor XIII A chain [KO:K03917] [EC:2.3.2.13]
110334054  F12; coagulation factor XII [KO:K01328] [EC:3.4.21.38]
110335652  C3; complement C3 [KO:K03990]
110335909  C4a; complement C4-A isoform X1 [KO:K03989]
110336125  Cfb; complement factor B isoform X1 [KO:K01335] [EC:3.4.21.47]
110336126  C2; complement C2 isoform X1 [KO:K01332] [EC:3.4.21.43]
110336411  Plat; tissue-type plasminogen activator [KO:K01343] [EC:3.4.21.68]
110336703  F10; coagulation factor X isoform X1 [KO:K01314] [EC:3.4.21.6]
110336811  Plaur; urokinase plasminogen activator surface receptor [KO:K03985]
110336817  Klkb1; plasma kallikrein [KO:K01324] [EC:3.4.21.34]
110336954  F7; coagulation factor VII [KO:K01320] [EC:3.4.21.21]
110337033  C5ar1; C5a anaphylatoxin chemotactic receptor 1 [KO:K04010]
110337055  F11; coagulation factor XI isoform X1 [KO:K01323] [EC:3.4.21.27]
110337063  F2rl3; proteinase-activated receptor 4 [KO:K04236]
110338193  Plg; plasminogen [KO:K01315] [EC:3.4.21.7]
110339172  Mbl2; mannose-binding protein C [KO:K03991]
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
mpah04611  Platelet activation
mpah04662  B cell receptor signaling pathway
mpah04750  Inflammatory mediator regulation of TRP channels
KO pathway
ko04610   

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