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
Drug
D00160  
Epsilon-Aminocaproic acid (JAN)
D00181  
Argatroban hydrate (JP16)
D01136  
Tranexamic acid (JP16/USAN/INN)
D01672  
Gabexate mesilate (JP16)
D01844  
Fondaparinux sodium (JAN/USAN/INN)
D01981  
Ximelagatran (JAN/USAN/INN)
D02112  
Heparin sodium (JP16/USP/INN)
D02956  
Anticoagulant heparin (USP)
D02971  
Aprotinin solution (JAN)
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 (genetical recombination) (JAN)
D03940  
Eculizumab (genetical recombination) (JAN)
D04029  
Razaxaban hydrochloride (USAN)
D04183  
Fidexaban (USAN/INN)
D04427  
Heparin calcium (JP16/USAN)
D04492  
Icatibant acetate (USAN)
D04977  
Parnaparin sodium (JP16)
D06398  
Tinzaparin sodium (USAN)
D06410  
Thrombomodulin alfa (genetical recombination) (JAN)
D06880  
Lepirudin (INN)
D07082  
Dabigatran etexilate methanesulfonate (JAN)
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
2161  
F12; coagulation factor XII (Hageman factor) [KO:K01328] [EC:3.4.21.38]
3818  
KLKB1; kallikrein B, plasma (Fletcher factor) 1 [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]
2160  
F11; coagulation factor XI [KO:K01323] [EC:3.4.21.27]
2158  
F9; coagulation factor IX [KO:K01321] [EC:3.4.21.22]
2157  
F8; coagulation factor VIII, procoagulant component [KO:K03899]
7450  
VWF; von Willebrand factor [KO:K03900]
2152  
F3; coagulation factor III (thromboplastin, tissue factor) [KO:K03901]
2155  
F7; coagulation factor VII (serum prothrombin conversion accelerator) [KO:K01320] [EC:3.4.21.21]
2159  
F10; coagulation factor X [KO:K01314] [EC:3.4.21.6]
2153  
F5; coagulation factor V (proaccelerin, labile factor) [KO:K03902]
2147  
F2; coagulation factor II (thrombin) [KO:K01313] [EC:3.4.21.5]
2243  
FGA; fibrinogen alpha chain [KO:K03903]
2244  
FGB; fibrinogen beta chain [KO:K03904]
2266  
FGG; fibrinogen gamma chain [KO:K03905]
2162  
F13A1; coagulation factor XIII, A1 polypeptide [KO:K03917] [EC:2.3.2.13]
2165  
F13B; coagulation factor XIII, B polypeptide [KO:K03906]
7056  
THBD; thrombomodulin [KO:K03907]
5624  
PROC; protein C (inactivator of coagulation factors Va and VIIIa) [KO:K01344] [EC:3.4.21.69]
5627  
PROS1; protein S (alpha) [KO:K03908]
5327  
PLAT; plasminogen activator, tissue [KO:K01343] [EC:3.4.21.68]
5328  
PLAU; plasminogen activator, urokinase [KO:K01348] [EC:3.4.21.73]
5340  
PLG; plasminogen [KO:K01315] [EC:3.4.21.7]
7035  
TFPI; tissue factor pathway inhibitor (lipoprotein-associated coagulation inhibitor) [KO:K03909]
3053  
SERPIND1; serpin peptidase inhibitor, clade D (heparin cofactor), member 1 [KO:K03912]
1361  
CPB2; carboxypeptidase B2 (plasma) [KO:K01300] [EC:3.4.17.20]
5054  
SERPINE1; serpin peptidase inhibitor, clade E (nexin, plasminogen activator inhibitor type 1), member 1 [KO:K03982]
5345  
SERPINF2; serpin peptidase inhibitor, clade F (alpha-2 antiplasmin, pigment epithelium derived factor), member 2 [KO:K03983]
462  
SERPINC1; serpin peptidase inhibitor, clade C (antithrombin), member 1 [KO:K03911]
5104  
SERPINA5; serpin peptidase inhibitor, clade A (alpha-1 antiproteinase, antitrypsin), member 5 [KO:K03913]
2  
A2M; alpha-2-macroglobulin [KO:K03910]
5265  
SERPINA1; serpin peptidase inhibitor, clade A (alpha-1 antiproteinase, antitrypsin), member 1 [KO:K03984]
2149  
F2R; coagulation factor II (thrombin) receptor [KO:K03914]
5329  
PLAUR; plasminogen activator, urokinase receptor [KO:K03985]
712  
C1QA; complement component 1, q subcomponent, A chain [KO:K03986]
713  
C1QB; complement component 1, q subcomponent, B chain [KO:K03987]
714  
C1QC; complement component 1, q subcomponent, C chain [KO:K03988]
715  
C1R; complement component 1, r subcomponent [KO:K01330] [EC:3.4.21.41]
716  
C1S; complement component 1, s subcomponent [KO:K01331] [EC:3.4.21.42]
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]
718  
C3; complement component 3 [KO:K03990]
629  
CFB; complement factor B [KO:K01335] [EC:3.4.21.47]
1675  
CFD; complement factor D (adipsin) [KO:K01334] [EC:3.4.21.46]
4153  
MBL2; mannose-binding lectin (protein C) 2, soluble [KO:K03991]
5648  
MASP1; mannan-binding lectin serine peptidase 1 (C4/C2 activating component of Ra-reactive factor) [KO:K03992] [EC:3.4.21.-]
10747  
MASP2; mannan-binding lectin serine peptidase 2 [KO:K03993] [EC:3.4.21.104]
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 polypeptide [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]
710  
SERPING1; serpin peptidase inhibitor, clade G (C1 inhibitor), member 1 [KO:K04001]
722  
C4BPA; complement component 4 binding protein, alpha [KO:K04002]
725  
C4BPB; complement component 4 binding protein, beta [KO:K04003]
3075  
CFH; complement factor H [KO:K04004]
3426  
CFI; complement factor I [KO:K01333] [EC:3.4.21.45]
1604  
CD55; CD55 molecule, decay accelerating factor for complement (Cromer blood group) [KO:K04006]
4179  
CD46; CD46 molecule, complement regulatory protein [KO:K04007]
966  
CD59; CD59 molecule, complement regulatory protein [KO:K04008]
719  
C3AR1; complement component 3a receptor 1 [KO:K04009]
728  
C5AR1; complement component 5a receptor 1 [KO:K04010]
1378  
CR1; complement component (3b/4b) receptor 1 (Knops blood group) [KO:K04011]
1380  
CR2; complement component (3d/Epstein Barr virus) receptor 2 [KO:K04012]
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)
KO pathway
 

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