Entry |
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Name |
Complement and coagulation cascades
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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.
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Class |
Organismal Systems; Immune system
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Pathway map |
map04610 | Complement and coagulation cascades |

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Disease |
H00983 | Alpha-2-plasmin inhibitor (a2-PI) deficiency |
H01078 | Fletcher factor deficiency |
H01103 | Alpha-1-antitrypsin deficiency |
H01381 | Antithrombin III deficiency |
H01434 | Atypical hemolytic uremic syndrome |
H02240 | Ehlers-Danlos syndrome periodontal type |
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Reference |
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Authors |
Bajic G, Degn SE, Thiel S, Andersen GR |
Title |
Complement activation, regulation, and molecular basis for complement-related diseases. |
Journal |
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Reference |
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Authors |
Mathern DR, Heeger PS |
Title |
Molecules Great and Small: The Complement System. |
Journal |
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Reference |
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Authors |
Merle NS, Church SE, Fremeaux-Bacchi V, Roumenina LT |
Title |
Complement System Part I - Molecular Mechanisms of Activation and Regulation. |
Journal |
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Reference |
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Authors |
Oikonomopoulou K, Ricklin D, Ward PA, Lambris JD |
Title |
Interactions between coagulation and complement--their role in inflammation. |
Journal |
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Reference |
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Authors |
Kurosawa S, Stearns-Kurosawa DJ |
Title |
Complement, thrombotic microangiopathy and disseminated intravascular coagulation. |
Journal |
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Reference |
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Authors |
Hillmeister P, Persson PB |
Title |
The Kallikrein-Kinin system. |
Journal |
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Reference |
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Authors |
Rau JC, Beaulieu LM, Huntington JA, Church FC |
Title |
Serpins in thrombosis, hemostasis and fibrinolysis. |
Journal |
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Reference |
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Authors |
Crooks MG, Hart SP |
Title |
Coagulation and anticoagulation in idiopathic pulmonary fibrosis. |
Journal |
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Reference |
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Authors |
Christiaans SC, Wagener BM, Esmon CT, Pittet JF |
Title |
Protein C and acute inflammation: a clinical and biological perspective. |
Journal |
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Reference |
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Authors |
Alberelli MA, De Candia E |
Title |
Functional role of protease activated receptors in vascular biology. |
Journal |
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Reference |
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Authors |
Smith HW, Marshall CJ |
Title |
Regulation of cell signalling by uPAR. |
Journal |
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Reference |
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Authors |
Ricklin D, Lambris JD |
Title |
Complement in immune and inflammatory disorders: pathophysiological mechanisms. |
Journal |
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Reference |
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Authors |
Ricklin D, Lambris JD |
Title |
Complement in immune and inflammatory disorders: therapeutic interventions. |
Journal |
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Reference |
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Authors |
Loof TG, Deicke C, Medina E |
Title |
The role of coagulation/fibrinolysis during Streptococcus pyogenes infection. |
Journal |
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Reference |
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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 |
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Related pathway |
map04662 | B cell receptor signaling pathway |
map04750 | Inflammatory mediator regulation of TRP channels |
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KO pathway |
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