KEGG   PATHWAY: ko04610Help
Entry
ko04610                     Pathway                                

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 system
BRITE hierarchy
Pathway map
Complement and coagulation cascades
ko04610

All organismsOrtholog table
Disease
H00102  
Classic complement pathway component defects
H00103  
Late complement pathway defects
H00104  
Alternative complement pathway component defects
H00105  
Mannose-binding lectin pathway component defects
H00106  
Complement regulatory protein defects
H00219  
Hemophilia
H00220  
Factor V deficiency
H00222  
Afibrinogenemia
H00223  
Inherited thrombophilia
H00845  
Familial amyloidosis
H00938  
Factor XI deficiency
H00941  
Factor XII deficiency
H00945  
Factor XIII deficiency
H00983  
Alpha-2-plasmin inhibitor (a2-PI) deficiency
H01006  
Hereditary angioedema
H01078  
Fletcher factor deficiency
H01103  
Alpha-1-antitrypsin (A1AT) deficiency
H01106  
Plasminogen activator inhibitor type 1 (PAI-1) deficiency
H01206  
Plasminogen deficiency
H01254  
Congenital prothrombin deficiency
H01381  
Antithrombin III deficiency
Orthology
K01328  
coagulation factor XII (Hageman factor) [EC:3.4.21.38]
K01324  
plasma kallikrein [EC:3.4.21.34]
K03898  
kininogen
K03915  
bradykinin receptor B1
K03916  
bradykinin receptor B2
K01323  
coagulation factor XI [EC:3.4.21.27]
K01321  
coagulation factor IX (Christmas factor) [EC:3.4.21.22]
K03899  
coagulation factor VIII
K03900  
von Willebrand factor
K03901  
coagulation factor III (tissue factor)
K01320  
coagulation factor VII [EC:3.4.21.21]
K01314  
coagulation factor X [EC:3.4.21.6]
K03902  
coagulation factor V (labile factor)
K01313  
coagulation factor II (thrombin) [EC:3.4.21.5]
K03903  
fibrinogen alpha chain
K03904  
fibrinogen beta chain
K03905  
fibrinogen gamma chain
K03917  
coagulation factor XIII A1 polypeptide [EC:2.3.2.13]
K03906  
coagulation factor XIII B polypeptide
K03907  
thrombomodulin
K01344  
protein C (activated) [EC:3.4.21.69]
K03908  
protein S
K01343  
tissue plasminogen activator [EC:3.4.21.68]
K01348  
urokinase plasminogen activator [EC:3.4.21.73]
K01315  
plasminogen [EC:3.4.21.7]
K03909  
tissue factor pathway inhibitor
K03912  
heparin cofactor II
K01300  
carboxypeptidase B2 [EC:3.4.17.20]
K03982  
plasminogen activator inhibitor-1
K03983  
alpha-2-antiplasmin
K03911  
antithrombin III
K03913  
protein C inhibitor
K03910  
alpha-2-macroglobulin
K03984  
alpha-1-antitrypsin
K03914  
coagulation factor II (thrombin) receptor
K03985  
plasminogen activator, urokinase receptor
K03986  
complement C1q subcomponent subunit A
K03987  
complement C1q subcomponent subunit B
K03988  
complement C1q subcomponent subunit C
K01330  
complement component 1, r subcomponent [EC:3.4.21.41]
K01331  
complement component 1, s subcomponent [EC:3.4.21.42]
K01332  
complement component 2 [EC:3.4.21.43]
K03989  
complement component 4
K03990  
complement component 3
K01335  
component factor B [EC:3.4.21.47]
K01334  
component factor D [EC:3.4.21.46]
K03991  
mannose-binding lectin
K03992  
mannan-binding lectin serine protease 1 [EC:3.4.21.-]
K03993  
mannan-binding lectin serine protease 2 [EC:3.4.21.104]
K03994  
complement component 5
K03995  
complement component 6
K03996  
complement component 7
K03997  
complement component 8 subunit alpha
K03998  
complement component 8 subunit beta
K03999  
complement component 8 subunit gamma
K04000  
complement component 9
K04001  
C1 inhibitor
K04002  
complement component 4 binding protein, alpha
K04003  
complement component 4 binding protein, beta
K04004  
complement factor H
K01333  
complement factor I [EC:3.4.21.45]
K04005  
complement receptor related protein
K04006  
decay accelerating factor
K04007  
membrane cofactor protein
K04008  
CD59 antigen
K04009  
C3a anaphylatoxin chemotactic receptor
K04010  
C5a anaphylatoxin chemotactic receptor
K04011  
complement component (3b/4b) receptor 1
K04012  
complement receptor type 2
Compound
C00290  
Fibrin
C00306  
Bradykinin
Reference
  Authors
Norris LA.
  Title
Blood coagulation.
  Journal
Best Pract Res Clin Obstet Gynaecol 17:369-83 (2003)
Reference
  Authors
Mann KG
  Title
Thrombin formation.
  Journal
Chest 124:4S-10S (2003)
Reference
  Authors
Bhole D, Stahl GL.
  Title
Therapeutic potential of targeting the complement cascade in critical care medicine.
  Journal
Crit Care Med 31:S97-104 (2003)
Reference
  Authors
Turnberg D, Botto M.
  Title
The regulation of the complement system: insights from genetically-engineered mice.
  Journal
Mol Immunol 40:145-53 (2003)
Reference
  Authors
Campbell DJ.
  Title
The kallikrein-kinin system in humans.
  Journal
Clin Exp Pharmacol Physiol 28:1060-5 (2001)
Reference
  Authors
Ueno A, Oh-ishi S.
  Title
Roles for the kallikrein-kinin system in inflammatory exudation and pain: lessons from studies on kininogen-deficient rats.
  Journal
J Pharmacol Sci 93:1-20 (2003)
Reference
  Authors
Ruf W, Dorfleutner A, Riewald M.
  Title
Specificity of coagulation factor signaling.
  Journal
J Thromb Haemost 1:1495-503 (2003)
Reference
  Authors
Mazar AP, Henkin J, Goldfarb RH.
  Title
The urokinase plasminogen activator system in cancer: implications for tumor angiogenesis and metastasis.
  Journal
Angiogenesis 3:15-32 (1999)
Reference
  Authors
Markovic SN, Inwards DJ, Frigas EA, Phyliky RP.
  Title
Acquired C1 esterase inhibitor deficiency.
  Journal
Ann Intern Med 132:144-50 (2000)
Reference
  Authors
Schmaier AH.
  Title
The plasma kallikrein-kinin system counterbalances the renin-angiotensin system.
  Journal
J Clin Invest 109:1007-9 (2002)
Reference
  Authors
Velasco-Velazquez MA, Barrera D, Gonzalez-Arenas A, Rosales C, Agramonte-Hevia J.
  Title
Macrophage--Mycobacterium tuberculosis interactions: role of complement receptor 3.
  Journal
Microb Pathog 35:125-31 (2003)
Reference
  Authors
Kim SJ, Gershov D, Ma X, Brot N, Elkon KB
  Title
Opsonization of apoptotic cells and its effect on macrophage and T cell immune responses.
  Journal
Ann N Y Acad Sci 987:68-78 (2003)
Reference
  Authors
Matsushita M, Thiel S, Jensenius JC, Terai I, Fujita T.
  Title
Proteolytic activities of two types of mannose-binding lectin-associated serine protease.
  Journal
J Immunol 165:2637-42 (2000)

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