KEGG   PATHWAY: hsa04610Help
Entry
hsa04610                    Pathway                                

Name
Complement and coagulation cascades - Homo sapiens (human)
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
hsa04610

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)
Drug
D00160  
Aminocaproic acid (USP/INN)
D00181  
Argatroban (USAN)
D01136  
Tranexamic acid (JP17/USAN/INN)
D01672  
Gabexate mesilate (JP17)
D01844  
Fondaparinux sodium (JAN/USAN/INN)
D01981  
Ximelagatran (JAN/USAN/INN)
D02112  
Heparin sodium (JP17/USP/INN)
D02956  
Anticoagulant heparin (USP)
D02971  
Aprotinin (USAN/INN)
D03136  
Bivalirudin (USAN/INN)
D03213  
Apixaban (JAN/USAN/INN)
D03337  
Reviparin sodium (JAN)
D03353  
Dalteparin sodium (JAN/USAN/INN)
D03644  
Danaparoid sodium (JAN/USAN)
D03674  
Enoxaparin sodium (JAN/USAN/INN)
D03692  
Desirudin (USAN/INN)
D03722  
Efegatran sulfate (USAN)
D03728  
Napsagatran (USAN)
D03931  
Ecallantide (USAN/INN)
D03940  
Eculizumab (USAN/INN)
D04029  
Razaxaban hydrochloride (USAN)
D04183  
Fidexaban (USAN/INN)
D04427  
Heparin calcium (JP17/USAN)
D04492  
Icatibant acetate (JAN/USAN)
D04977  
Parnaparin sodium (JP17)
D06398  
Tinzaparin sodium (USAN)
D06410  
Thrombomodulin alfa (genetical recombination) (JAN)
D06880  
Lepirudin (INN)
D07082  
Dabigatran etexilate mesylate (USAN)
D07086  
Rivaroxaban (JAN/USAN/INN)
D07143  
Melagatran (INN)
D07144  
Dabigatran etexilate (USAN/INN)
D07510  
Adomiparin (USAN)
D08004  
Gabexate (INN)
D08656  
Aleplasinin (USAN/INN)
D08873  
Betrixaban (USAN)
D08913  
Eribaxaban (USAN)
D09546  
Edoxaban tosilate hydrate (JAN)
D09707  
Dabigatran (USAN/INN)
D09710  
Edoxaban (USAN/INN)
D09765  
Vorapaxar (USAN/INN)
D09766  
Vorapaxar sulfate (JAN/USAN)
D09817  
Darexaban maleate (JAN)
D09860  
Adomiparin sodium (USAN)
D09866  
Atopaxar (USAN)
D09867  
Atopaxar hydrobromide (USAN)
D09923  
Letaxaban (USAN/INN)
D10023  
Pexelizumab (USAN/INN)
D10150  
Pegnivacogin sodium (USAN)
D10418  
Delparantag pentahydrochloride (USAN)
D10471  
Idraparinux sodium (USAN)
D10484  
Pegnivacogin (USAN)
Organism
Homo sapiens (human) [GN:hsa]
Gene
2152  
F3; coagulation factor III, tissue factor [KO:K03901]
2155  
F7; coagulation factor VII [KO:K01320] [EC:3.4.21.21]
2159  
F10; coagulation factor X [KO:K01314] [EC:3.4.21.6]
2153  
F5; coagulation factor V [KO:K03902]
2147  
F2; coagulation factor II, thrombin [KO:K01313] [EC:3.4.21.5]
2161  
F12; coagulation factor XII [KO:K01328] [EC:3.4.21.38]
2160  
F11; coagulation factor XI [KO:K01323] [EC:3.4.21.27]
2158  
F9; coagulation factor IX [KO:K01321] [EC:3.4.21.22]
7450  
VWF; von Willebrand factor [KO:K03900]
2157  
F8; coagulation factor VIII [KO:K03899]
7056  
THBD; thrombomodulin [KO:K03907]
10544  
PROCR; protein C receptor [KO:K06557]
5624  
PROC; protein C, inactivator of coagulation factors Va and VIIIa [KO:K01344] [EC:3.4.21.69]
2149  
F2R; coagulation factor II thrombin receptor [KO:K03914]
2151  
F2RL2; coagulation factor II thrombin receptor like 2 [KO:K04235]
9002  
F2RL3; F2R like thrombin/trypsin receptor 3 [KO:K04236]
2162  
F13A1; coagulation factor XIII A chain [KO:K03917] [EC:2.3.2.13]
2165  
F13B; coagulation factor XIII B chain [KO:K03906]
1361  
CPB2; carboxypeptidase B2 [KO:K01300] [EC:3.4.17.20]
2243  
FGA; fibrinogen alpha chain [KO:K03903]
2244  
FGB; fibrinogen beta chain [KO:K03904]
2266  
FGG; fibrinogen gamma chain [KO:K03905]
3818  
KLKB1; kallikrein B1 [KO:K01324] [EC:3.4.21.34]
3827  
KNG1; kininogen 1 [KO:K03898]
623  
BDKRB1; bradykinin receptor B1 [KO:K03915]
624  
BDKRB2; bradykinin receptor B2 [KO:K03916]
5340  
PLG; plasminogen [KO:K01315] [EC:3.4.21.7]
7035  
TFPI; tissue factor pathway inhibitor [KO:K03909]
462  
SERPINC1; serpin family C member 1 [KO:K03911]
3053  
SERPIND1; serpin family D member 1 [KO:K03912]
5104  
SERPINA5; serpin family A member 5 [KO:K03913]
5627  
PROS1; protein S (alpha) [KO:K03908]
5054  
SERPINE1; serpin family E member 1 [KO:K03982]
5055  
SERPINB2; serpin family B member 2 [KO:K19821]
5327  
PLAT; plasminogen activator, tissue type [KO:K01343] [EC:3.4.21.68]
5328  
PLAU; plasminogen activator, urokinase [KO:K01348] [EC:3.4.21.73]
5329  
PLAUR; plasminogen activator, urokinase receptor [KO:K03985]
5265  
SERPINA1; serpin family A member 1 [KO:K03984]
5345  
SERPINF2; serpin family F member 2 [KO:K03983]
2  
A2M; alpha-2-macroglobulin [KO:K03910]
629  
CFB; complement factor B [KO:K01335] [EC:3.4.21.47]
1675  
CFD; complement factor D [KO:K01334] [EC:3.4.21.46]
718  
C3; complement component 3 [KO:K03990]
727  
C5; complement component 5 [KO:K03994]
729  
C6; complement component 6 [KO:K03995]
730  
C7; complement component 7 [KO:K03996]
731  
C8A; complement component 8 alpha subunit [KO:K03997]
732  
C8B; complement component 8, beta polypeptide [KO:K03998]
733  
C8G; complement component 8, gamma polypeptide [KO:K03999]
735  
C9; complement component 9 [KO:K04000]
712  
C1QA; complement C1q A chain [KO:K03986]
713  
C1QB; complement C1q B chain [KO:K03987]
714  
C1QC; complement C1q C chain [KO:K03988]
715  
C1R; complement C1r subcomponent [KO:K01330] [EC:3.4.21.41]
716  
C1S; complement component 1, s subcomponent [KO:K01331] [EC:3.4.21.42]
4153  
MBL2; mannose binding lectin 2 [KO:K03991]
5648  
MASP1; mannan binding lectin serine peptidase 1 [KO:K03992] [EC:3.4.21.-]
10747  
MASP2; mannan binding lectin serine peptidase 2 [KO:K03993] [EC:3.4.21.104]
717  
C2; complement component 2 [KO:K01332] [EC:3.4.21.43]
720  
C4A; complement component 4A (Rodgers blood group) [KO:K03989]
721  
C4B; complement component 4B (Chido blood group) [KO:K03989]
719  
C3AR1; complement component 3a receptor 1 [KO:K04009]
11326  
VSIG4; V-set and immunoglobulin domain containing 4 [KO:K19822]
1378  
CR1; complement component 3b/4b receptor 1 (Knops blood group) [KO:K04011]
1380  
CR2; complement component 3d receptor 2 [KO:K04012]
3684  
ITGAM; integrin subunit alpha M [KO:K06461]
3689  
ITGB2; integrin subunit beta 2 [KO:K06464]
3687  
ITGAX; integrin subunit alpha X [KO:K06462]
728  
C5AR1; complement component 5a receptor 1 [KO:K04010]
3075  
CFH; complement factor H [KO:K04004]
3426  
CFI; complement factor I [KO:K01333] [EC:3.4.21.45]
710  
SERPING1; serpin family G member 1 [KO:K04001]
1604  
CD55; CD55 molecule (Cromer blood group) [KO:K04006]
4179  
CD46; CD46 molecule [KO:K04007]
722  
C4BPA; complement component 4 binding protein alpha [KO:K04002]
725  
C4BPB; complement component 4 binding protein beta [KO:K04003]
966  
CD59; CD59 molecule [KO:K04008]
1191  
CLU; clusterin [KO:K17252]
7448  
VTN; vitronectin [KO:K06251]
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)
KO pathway
 

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