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
H01434  
Atypical hemolytic uremic syndrome (aHUS)
Orthology
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]
K01328  
coagulation factor XII (Hageman factor) [EC:3.4.21.38]
K01323  
coagulation factor XI [EC:3.4.21.27]
K01321  
coagulation factor IX (Christmas factor) [EC:3.4.21.22]
K03900  
von Willebrand factor
K03899  
coagulation factor VIII
K03907  
thrombomodulin
K06557  
protein C receptor, endothelial (EPCR)
K01344  
protein C (activated) [EC:3.4.21.69]
K03914  
coagulation factor II (thrombin) receptor
K04235  
coagulation factor II (thrombin) receptor-like 2
K04236  
coagulation factor II (thrombin) receptor-like 3
K03917  
coagulation factor XIII A1 polypeptide [EC:2.3.2.13]
K03906  
coagulation factor XIII B polypeptide
K01300  
carboxypeptidase B2 [EC:3.4.17.20]
K03903  
fibrinogen alpha chain
K03904  
fibrinogen beta chain
K03905  
fibrinogen gamma chain
K01324  
plasma kallikrein [EC:3.4.21.34]
K03898  
kininogen
K03915  
bradykinin receptor B1
K03916  
bradykinin receptor B2
K01315  
plasminogen [EC:3.4.21.7]
K03909  
tissue factor pathway inhibitor
K03911  
antithrombin III
K03912  
heparin cofactor II
K03913  
protein C inhibitor
K03908  
protein S
K03982  
plasminogen activator inhibitor 1
K19821  
plasminogen activator inhibitor 2
K01343  
tissue plasminogen activator [EC:3.4.21.68]
K01348  
urokinase plasminogen activator [EC:3.4.21.73]
K03985  
plasminogen activator, urokinase receptor
K03984  
alpha-1-antitrypsin
K03983  
alpha-2-antiplasmin
K03910  
alpha-2-macroglobulin
K01335  
component factor B [EC:3.4.21.47]
K01334  
component factor D [EC:3.4.21.46]
K03990  
complement component 3
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
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]
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]
K01332  
complement component 2 [EC:3.4.21.43]
K03989  
complement component 4
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
K06461  
integrin alpha M
K06464  
integrin beta 2
K06462  
integrin alpha X
K04010  
C5a anaphylatoxin chemotactic receptor
K04004  
complement factor H
K01333  
complement factor I [EC:3.4.21.45]
K04001  
C1 inhibitor
K04006  
decay accelerating factor
K04007  
membrane cofactor protein
K04002  
complement component 4 binding protein, alpha
K04003  
complement component 4 binding protein, beta
K04008  
CD59 antigen
K17252  
clusterin
K06251  
vitronectin
Compound
C00290  
Fibrin
C00306  
Bradykinin
Reference
  Authors
Bajic G, Degn SE, Thiel S, Andersen GR
  Title
Complement activation, regulation, and molecular basis for complement-related diseases.
  Journal
EMBO J 34:2735-57 (2015)
Reference
  Authors
Mathern DR, Heeger PS
  Title
Molecules Great and Small: The Complement System.
  Journal
Clin J Am Soc Nephrol 10:1636-50 (2015)
Reference
  Authors
Merle NS, Church SE, Fremeaux-Bacchi V, Roumenina LT
  Title
Complement System Part I - Molecular Mechanisms of Activation and Regulation.
  Journal
Front Immunol 6:262 (2015)
Reference
  Authors
Oikonomopoulou K, Ricklin D, Ward PA, Lambris JD
  Title
Interactions between coagulation and complement--their role in inflammation.
  Journal
Semin Immunopathol 34:151-65 (2012)
Reference
  Authors
Kurosawa S, Stearns-Kurosawa DJ
  Title
Complement, thrombotic microangiopathy and disseminated intravascular coagulation.
  Journal
J Intensive Care 2:65 (2014)
Reference
  Authors
Hillmeister P, Persson PB
  Title
The Kallikrein-Kinin system.
  Journal
Acta Physiol (Oxf) 206:215-9 (2012)
Reference
  Authors
Rau JC, Beaulieu LM, Huntington JA, Church FC
  Title
Serpins in thrombosis, hemostasis and fibrinolysis.
  Journal
J Thromb Haemost 5 Suppl 1:102-15 (2007)
Reference
  Authors
Crooks MG, Hart SP
  Title
Coagulation and anticoagulation in idiopathic pulmonary fibrosis.
  Journal
Eur Respir Rev 24:392-9 (2015)
Reference
  Authors
Christiaans SC, Wagener BM, Esmon CT, Pittet JF
  Title
Protein C and acute inflammation: a clinical and biological perspective.
  Journal
Am J Physiol Lung Cell Mol Physiol 305:L455-66 (2013)
Reference
  Authors
Alberelli MA, De Candia E
  Title
Functional role of protease activated receptors in vascular biology.
  Journal
Vascul Pharmacol 62:72-81 (2014)
Reference
  Authors
Smith HW, Marshall CJ
  Title
Regulation of cell signalling by uPAR.
  Journal
Nat Rev Mol Cell Biol 11:23-36 (2010)
Reference
  Authors
Ricklin D, Lambris JD
  Title
Complement in immune and inflammatory disorders: pathophysiological mechanisms.
  Journal
J Immunol 190:3831-8 (2013)
Reference
  Authors
Ricklin D, Lambris JD
  Title
Complement in immune and inflammatory disorders: therapeutic interventions.
  Journal
J Immunol 190:3839-47 (2013)
Reference
  Authors
Loof TG, Deicke C, Medina E
  Title
The role of coagulation/fibrinolysis during Streptococcus pyogenes infection.
  Journal
Front Cell Infect Microbiol 4:128 (2014)

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