Entry Name Complement and coagulation cascades
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 systemBRITE hierarchy
Pathway map ko04610 Complement and coagulation cascades
Orthology K03899 F8; coagulation factor VIII
K03900 VWF; von Willebrand factor
K03901 F3, CD142; coagulation factor III (tissue factor)
K03902 F5; coagulation factor V (labile factor)
K03903 FGA; fibrinogen alpha chain
K03904 FGB; fibrinogen beta chain
K03905 FGG; fibrinogen gamma chain
K03906 F13B; coagulation factor XIII B polypeptide
K03907 THBD, CD141; thrombomodulin
K03909 TFPI; tissue factor pathway inhibitor
K03910 A2M; alpha-2-macroglobulin
K03911 SERPINC1, AT3; antithrombin III
K03912 SERPIND1, HCF2; heparin cofactor II
K03913 SERPINA5, PCI; protein C inhibitor
K03914 F2R, PAR1; coagulation factor II (thrombin) receptor
K03915 BDKRB1; bradykinin receptor B1
K03916 BDKRB2; bradykinin receptor B2
K03982 SERPINE1, PAI1; plasminogen activator inhibitor 1
K03983 SERPINF2, AAP; alpha-2-antiplasmin
K03984 SERPINA1, AAT; alpha-1-antitrypsin
K03985 PLAUR, CD87; plasminogen activator, urokinase receptor
K03986 C1QA; complement C1q subcomponent subunit A
K03987 C1QB; complement C1q subcomponent subunit B
K03988 C1QG; complement C1q subcomponent subunit C
K03989 C4; complement component 4
K03990 C3; complement component 3
K03991 MBL; mannose-binding lectin
K03992 MASP1; mannan-binding lectin serine protease 1 [EC:3.4.21.-]
K03994 C5; complement component 5
K03995 C6; complement component 6
K03996 C7; complement component 7
K03997 C8A; complement component 8 subunit alpha
K03998 C8B; complement component 8 subunit beta
K03999 C8G; complement component 8 subunit gamma
K04000 C9; complement component 9
K04001 SERPING1, C1INH; C1 inhibitor
K04002 C4BPA; complement component 4 binding protein alpha
K04003 C4BPB; complement component 4 binding protein beta
K04004 CFH, HF1; complement factor H
K04006 DAF, CD55; decay accelerating factor
K04007 CD46, MCP; membrane cofactor protein
K04009 C3AR1; C3a anaphylatoxin chemotactic receptor
K04010 C5AR1, CD88; C5a anaphylatoxin chemotactic receptor 1
K04011 CR1, CD35; complement component (3b/4b) receptor 1
K04012 CR2, CD21; complement receptor type 2
K04235 F2RL2, PAR3; coagulation factor II (thrombin) receptor-like 2
K04236 F2RL3, PAR4; coagulation factor II (thrombin) receptor-like 3
K06461 ITGAM, CD11b; integrin alpha M
K06462 ITGAX, CD11c; integrin alpha X
K06464 ITGB2, CD18; integrin beta 2
K06557 PROCR, CD201; protein C receptor, endothelial (EPCR)
K16643 SERPINE2; glia-derived nexin
K19821 SERPINB2, PAI2; plasminogen activator inhibitor 2
K19822 VSIG4, CRIg; V-set and immunoglobulin domain-containing protein 4
K23815 CFHR1_2_3; complement factor H-related protein 1/2/3
K23816 CFHR4; complement factor H-related protein 4
K23817 CFHR5; complement factor H-related protein 5
Compound C03378 Coagulation factor VIIa
C03379 Coagulation factor XIIa
C04001 Coagulation factor VIIIa
C22531 Complement component 2a
C22532 Complement component 2b
C22533 Complement component 3b
C22534 Complement component 3f
C22536 Complement component 3dg
C22537 Complement component 3c
C22538 Complement component 3d
C22541 Complement component 4a
C22542 Complement component 4b
C22543 Complement component 4c
C22544 Complement component 4d
C22545 Complement component 5b
C22546 Coagulation factor XIIIa
Reference Authors Bajic G, Degn SE, Thiel S, Andersen GR
Title Complement activation, regulation, and molecular basis for complement-related diseases.
Journal Reference Authors Mathern DR, Heeger PS
Title Molecules Great and Small: The Complement System.
Journal Reference Authors Merle NS, Church SE, Fremeaux-Bacchi V, Roumenina LT
Title Complement System Part I - Molecular Mechanisms of Activation and Regulation.
Journal Reference Authors Oikonomopoulou K, Ricklin D, Ward PA, Lambris JD
Title Interactions between coagulation and complement--their role in inflammation.
Journal Reference Authors Kurosawa S, Stearns-Kurosawa DJ
Title Complement, thrombotic microangiopathy and disseminated intravascular coagulation.
Journal Reference Authors Hillmeister P, Persson PB
Title The Kallikrein-Kinin system.
Journal Reference Authors Rau JC, Beaulieu LM, Huntington JA, Church FC
Title Serpins in thrombosis, hemostasis and fibrinolysis.
Journal Reference Authors Crooks MG, Hart SP
Title Coagulation and anticoagulation in idiopathic pulmonary fibrosis.
Journal Reference Authors Christiaans SC, Wagener BM, Esmon CT, Pittet JF
Title Protein C and acute inflammation: a clinical and biological perspective.
Journal Reference Authors Alberelli MA, De Candia E
Title Functional role of protease activated receptors in vascular biology.
Journal Reference Authors Smith HW, Marshall CJ
Title Regulation of cell signalling by uPAR.
Journal Reference Authors Ricklin D, Lambris JD
Title Complement in immune and inflammatory disorders: pathophysiological mechanisms.
Journal Reference Authors Ricklin D, Lambris JD
Title Complement in immune and inflammatory disorders: therapeutic interventions.
Journal Reference Authors Loof TG, Deicke C, Medina E
Title The role of coagulation/fibrinolysis during Streptococcus pyogenes infection.
Journal 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 Related ko04662 B cell receptor signaling pathway
ko04750 Inflammatory mediator regulation of TRP channels
