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
Ortholog table Orthology K03901 coagulation factor III (tissue factor)
K03899 coagulation factor VIII
K03902 coagulation factor V (labile factor)
K03906 coagulation factor XIII B polypeptide
K03914 coagulation factor II (thrombin) receptor
K04235 coagulation factor II (thrombin) receptor-like 2
K04236 coagulation factor II (thrombin) receptor-like 3
K06557 protein C receptor, endothelial (EPCR)
K03982 plasminogen activator inhibitor 1
K19821 plasminogen activator inhibitor 2
K03985 plasminogen activator, urokinase receptor
K03909 tissue factor pathway inhibitor
K03986 complement C1q subcomponent subunit A
K03987 complement C1q subcomponent subunit B
K03988 complement C1q subcomponent subunit C
K03992 mannan-binding lectin serine protease 1 [EC:3.4.21.-]
K03997 complement component 8 subunit alpha
K03998 complement component 8 subunit beta
K03999 complement component 8 subunit gamma
K04009 C3a anaphylatoxin chemotactic receptor
K19822 V-set and immunoglobulin domain-containing protein 4
K04011 complement component (3b/4b) receptor 1
K04012 complement receptor type 2
K04010 C5a anaphylatoxin chemotactic receptor 1
K04002 complement component 4 binding protein alpha
K04003 complement component 4 binding protein beta
K04007 membrane cofactor protein
K04006 decay accelerating factor
K23815 complement factor H-related protein 1/2/3
K23816 complement factor H-related protein 4
K23817 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
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