| 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 |
| Complement and coagulation cascades |
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| Disease |
| Classic complement pathway component defects | | Late complement pathway defects | | Alternative complement pathway component defects | | Mannose-binding lectin pathway component defects | | Complement regulatory protein defects | | Hemophilia | | Factor V deficiency | | Afibrinogenemia | | Inherited thrombophilia | | Familial amyloidosis | | Factor XI deficiency | | Factor XII deficiency | | Factor XIII deficiency | | Alpha-2-plasmin inhibitor (a2-PI) deficiency | | Hereditary angioedema | | Fletcher factor deficiency | | Alpha-1-antitrypsin (A1AT) deficiency | | Plasminogen activator inhibitor type 1 (PAI-1) deficiency | | Plasminogen deficiency | | Congenital prothrombin deficiency |
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| Reference |
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| Authors |
Norris LA. |
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| Title |
Blood coagulation. |
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| Journal |
Best Pract Res Clin Obstet Gynaecol 17:369-83 (2003) |
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| Reference |
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| Authors |
Mann KG. |
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| Title |
Thrombin formation. |
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| Journal |
Chest 124:4S-10S (2003) |
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| Reference |
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| Authors |
Bhole D, Stahl GL. |
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| Title |
Therapeutic potential of targeting the complement cascade in critical care medicine. |
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| Journal |
Crit Care Med 31:S97-104 (2003) |
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| Reference |
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| Authors |
Turnberg D, Botto M. |
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| Title |
The regulation of the complement system: insights from genetically-engineered mice. |
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| Journal |
Mol Immunol 40:145-53 (2003) |
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| Reference |
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| Authors |
Campbell DJ. |
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| Title |
The kallikrein-kinin system in humans. |
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| Journal |
Clin Exp Pharmacol Physiol 28:1060-5 (2001) |
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| Reference |
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| Authors |
Ueno A, Oh-ishi S. |
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| Title |
Roles for the kallikrein-kinin system in inflammatory exudation and pain: lessons from studies on kininogen-deficient rats. |
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| Journal |
J Pharmacol Sci 93:1-20 (2003) |
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| Reference |
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| Authors |
Ruf W, Dorfleutner A, Riewald M. |
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| Title |
Specificity of coagulation factor signaling. |
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| Journal |
J Thromb Haemost 1:1495-503 (2003) |
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| Reference |
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| Authors |
Mazar AP, Henkin J, Goldfarb RH. |
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| Title |
The urokinase plasminogen activator system in cancer: implications for tumor angiogenesis and metastasis. |
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| Journal |
Angiogenesis 3:15-32 (1999) |
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| Reference |
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| Authors |
Markovic SN, Inwards DJ, Frigas EA, Phyliky RP. |
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| Title |
Acquired C1 esterase inhibitor deficiency. |
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| Journal |
Ann Intern Med 132:144-50 (2000) |
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| Reference |
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| Authors |
Schmaier AH. |
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| Title |
The plasma kallikrein-kinin system counterbalances the renin-angiotensin system. |
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| Journal |
J Clin Invest 109:1007-9 (2002) |
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| Reference |
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| Authors |
Velasco-Velazquez MA, Barrera D, Gonzalez-Arenas A, Rosales C, Agramonte-Hevia J. |
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| Title |
Macrophage--Mycobacterium tuberculosis interactions: role of complement receptor 3. |
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| Journal |
Microb Pathog 35:125-31 (2003) |
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| Reference |
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| Authors |
Kim SJ, Gershov D, Ma X, Brot N, Elkon KB. |
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| Title |
Opsonization of apoptotic cells and its effect on macrophage and T cell immune responses. |
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| Journal |
Ann N Y Acad Sci 987:68-78 (2003) |
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| Reference |
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| Authors |
Matsushita M, Thiel S, Jensenius JC, Terai I, Fujita T. |
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| Title |
Proteolytic activities of two types of mannose-binding lectin-associated serine protease. |
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| Journal |
J Immunol 165:2637-42 (2000) |
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| KO pathway |
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