KEGG   PATHWAY: mpah04610
mpah04610                   Pathway                                
Complement and coagulation cascades - Mus pahari (shrew mouse)
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.
Organismal Systems; Immune system
Pathway map
mpah04610  Complement and coagulation cascades

Mus pahari (shrew mouse) [GN:mpah]
110320545  F3; tissue factor [KO:K03901]
110336954  F7; coagulation factor VII [KO:K01320] [EC:]
110336703  F10; coagulation factor X isoform X1 [KO:K01314] [EC:]
110322113  F5; coagulation factor V [KO:K03902]
110319115  F2; prothrombin isoform X1 [KO:K01313] [EC:]
110334054  F12; coagulation factor XII [KO:K01328] [EC:]
110337055  F11; coagulation factor XI isoform X1 [KO:K01323] [EC:]
110314135  F9; coagulation factor IX [KO:K01321] [EC:]
110316106  Vwf; von Willebrand factor [KO:K03900]
110313611  F8; coagulation factor VIII isoform X1 [KO:K03899]
110318522  Thbd; thrombomodulin [KO:K03907]
110319235  Procr; endothelial protein C receptor [KO:K06557]
110333316  Proc; vitamin K-dependent protein C isoform X1 [KO:K01344] [EC:]
110328985  F2r; proteinase-activated receptor 1 [KO:K03914]
110328865  F2rl2; proteinase-activated receptor 3 [KO:K04235]
110337063  F2rl3; proteinase-activated receptor 4 [KO:K04236]
110333906  F13a1; coagulation factor XIII A chain [KO:K03917] [EC:]
110322312  coagulation factor XIII B chain [KO:K03906]
110325644  Cpb2; carboxypeptidase B2 [KO:K01300] [EC:]
110321050  Fga; fibrinogen alpha chain isoform X1 [KO:K03903]
110320684  Fgb; fibrinogen beta chain isoform X1 [KO:K03904]
110320605  Fgg; fibrinogen gamma chain isoform X1 [KO:K03905]
110336817  Klkb1; plasma kallikrein [KO:K01324] [EC:]
110329894  Kng1; kininogen-1 [KO:K03898]
110330025  kininogen-1-like [KO:K03898]
110325005  Bdkrb1; B1 bradykinin receptor [KO:K03915]
110324855  Bdkrb2; B2 bradykinin receptor isoform X1 [KO:K03916]
110338193  Plg; plasminogen [KO:K01315] [EC:]
110318900  Tfpi; tissue factor pathway inhibitor isoform X1 [KO:K03909]
110322401  Serpinc1; antithrombin-III [KO:K03911]
110329478  Serpind1; heparin cofactor 2 [KO:K03912]
110324828  Serpina5; plasma serine protease inhibitor [KO:K03913]
110329814  Pros1; vitamin K-dependent protein S [KO:K03908]
110313068  Serpine1; plasminogen activator inhibitor 1 [KO:K03982]
110322355  Serpinb2; plasminogen activator inhibitor 2 [KO:K19821]
110336411  Plat; tissue-type plasminogen activator [KO:K01343] [EC:]
110325641  Plau; urokinase-type plasminogen activator [KO:K01348] [EC:]
110336811  Plaur; urokinase plasminogen activator surface receptor [KO:K03985]
110324788  alpha-1-antitrypsin 1-6 isoform X1 [KO:K03984]
110324428  alpha-1-antitrypsin 1-3 isoform X1 [KO:K03984]
110324489  alpha-1-antitrypsin 1-3-like [KO:K03984]
110332409  Serpinf2; alpha-2-antiplasmin isoform X1 [KO:K03983]
110316081  A2m; alpha-2-macroglobulin [KO:K03910]
110336125  Cfb; complement factor B isoform X1 [KO:K01335] [EC:]
110326960  Cfd; complement factor D isoform X1 [KO:K01334] [EC:]
110335652  C3; complement C3 [KO:K03990]
110317539  C5; complement C5 [KO:K03994]
110329165  C6; complement component C6 [KO:K03995]
110328891  C7; complement component C7 [KO:K03996]
110323615  C8a; complement component C8 alpha chain [KO:K03997]
110323447  C8b; complement component C8 beta chain isoform X1 [KO:K03998]
110318150  C8g; complement component C8 gamma chain [KO:K03999]
110329162  C9; complement component C9 [KO:K04000]
110323698  C1qa; complement C1q subcomponent subunit A [KO:K03986]
110323604  C1qb; complement C1q subcomponent subunit B [KO:K03987]
110323611  C1qc; complement C1q subcomponent subunit C [KO:K03988]
110314999  complement C1r-A subcomponent-like [KO:K01330] [EC:]
110316472  C1r; complement C1r subcomponent [KO:K01330] [EC:]
110314998  complement C1s subcomponent-like [KO:K01331] [EC:]
110316909  C1s; complement C1s subcomponent [KO:K01331] [EC:]
110339172  Mbl2; mannose-binding protein C [KO:K03991]
110325947  mannose-binding protein A [KO:K03991]
110329505  Masp1; mannan-binding lectin serine protease 1 [KO:K03992] [EC:3.4.21.-]
110323473  Masp2; mannan-binding lectin serine protease 2 isoform X1 [KO:K03993] [EC:]
110336126  C2; complement C2 isoform X1 [KO:K01332] [EC:]
110335909  C4a; complement C4-A isoform X1 [KO:K03989]
110317061  C3ar1; C3a anaphylatoxin chemotactic receptor [KO:K04009]
110314138  Vsig4; V-set and immunoglobulin domain-containing protein 4 [KO:K19822]
110321621  Cr1l; complement component receptor 1-like protein isoform X1 [KO:K04011]
110321620  Cr2; complement receptor type 2 [KO:K04012]
110327528  Itgam; integrin alpha-M isoform X1 [KO:K06461]
110326926  Itgb2; integrin beta-2 [KO:K06464]
110330040  LOW QUALITY PROTEIN: integrin beta-2-like protein [KO:K06464]
110327271  Itgax; integrin alpha-X [KO:K06462]
110337033  C5ar1; C5a anaphylatoxin chemotactic receptor 1 [KO:K04010]
110322014  Cfh; complement factor H [KO:K04004]
110320634  Cfi; complement factor I isoform X1 [KO:K01333] [EC:]
110318833  Serping1; plasma protease C1 inhibitor [KO:K04001]
110322166  Cd55; complement decay-accelerating factor isoform X1 [KO:K04006]
110322354  Cd46; membrane cofactor protein [KO:K04007]
110322103  C4bpa; C4b-binding protein alpha chain [KO:K04002]
110322001  C4bpb; LOW QUALITY PROTEIN: C4b-binding protein beta chain [KO:K04003]
110315756  complement factor H-related protein 4-like [KO:K23815]
110319661  CD59A glycoprotein-like [KO:K04008]
110319701  Cd59; CD59 glycoprotein [KO:K04008]
110325498  Clu; clusterin [KO:K17252]
110332069  Vtn; vitronectin [KO:K06251]
C00290  Fibrin
Bajic G, Degn SE, Thiel S, Andersen GR
Complement activation, regulation, and molecular basis for complement-related diseases.
EMBO J 34:2735-57 (2015)
Mathern DR, Heeger PS
Molecules Great and Small: The Complement System.
Clin J Am Soc Nephrol 10:1636-50 (2015)
Merle NS, Church SE, Fremeaux-Bacchi V, Roumenina LT
Complement System Part I - Molecular Mechanisms of Activation and Regulation.
Front Immunol 6:262 (2015)
Oikonomopoulou K, Ricklin D, Ward PA, Lambris JD
Interactions between coagulation and complement--their role in inflammation.
Semin Immunopathol 34:151-65 (2012)
Kurosawa S, Stearns-Kurosawa DJ
Complement, thrombotic microangiopathy and disseminated intravascular coagulation.
J Intensive Care 2:65 (2014)
Hillmeister P, Persson PB
The Kallikrein-Kinin system.
Acta Physiol (Oxf) 206:215-9 (2012)
Rau JC, Beaulieu LM, Huntington JA, Church FC
Serpins in thrombosis, hemostasis and fibrinolysis.
J Thromb Haemost 5 Suppl 1:102-15 (2007)
Crooks MG, Hart SP
Coagulation and anticoagulation in idiopathic pulmonary fibrosis.
Eur Respir Rev 24:392-9 (2015)
Christiaans SC, Wagener BM, Esmon CT, Pittet JF
Protein C and acute inflammation: a clinical and biological perspective.
Am J Physiol Lung Cell Mol Physiol 305:L455-66 (2013)
Alberelli MA, De Candia E
Functional role of protease activated receptors in vascular biology.
Vascul Pharmacol 62:72-81 (2014)
Smith HW, Marshall CJ
Regulation of cell signalling by uPAR.
Nat Rev Mol Cell Biol 11:23-36 (2010)
Ricklin D, Lambris JD
Complement in immune and inflammatory disorders: pathophysiological mechanisms.
J Immunol 190:3831-8 (2013)
Ricklin D, Lambris JD
Complement in immune and inflammatory disorders: therapeutic interventions.
J Immunol 190:3839-47 (2013)
Loof TG, Deicke C, Medina E
The role of coagulation/fibrinolysis during Streptococcus pyogenes infection.
Front Cell Infect Microbiol 4:128 (2014)
Jozsi M, Tortajada A, Uzonyi B, Goicoechea de Jorge E, Rodriguez de Cordoba S
Factor H-related proteins determine complement-activating surfaces.
Trends Immunol 36:374-84 (2015)
mpah04611  Platelet activation
mpah04662  B cell receptor signaling pathway
mpah04750  Inflammatory mediator regulation of TRP channels
KO pathway

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