KEGG   PATHWAY: rno04610
Entry
rno04610                    Pathway                                
Name
Complement and coagulation cascades - Rattus norvegicus (rat)
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
Pathway map
rno04610  Complement and coagulation cascades
rno04610

Organism
Rattus norvegicus (rat) [GN:rno]
Gene
102554977  Cfhr1-ps1; LOW QUALITY PROTEIN: complement factor H-related protein 1-like [KO:K23815]
113936  Cpb2; carboxypeptidase B2 precursor [KO:K01300] [EC:3.4.17.20]
113959  C5ar1; C5a anaphylatoxin chemotactic receptor 1 [KO:K04010]
116498  F2rl3; proteinase-activated receptor 4 [KO:K04236]
116669  Vwf; von Willebrand factor precursor [KO:K03900]
117512  C9; complement component C9 [KO:K04000]
117517  C7; complement component C7 isoform X1 [KO:K03996]
155012  Cfh; complement factor H precursor [KO:K04004]
192262  C1s; complement C1s subcomponent precursor [KO:K01331] [EC:3.4.21.42]
24153  A2m; alpha-2-macroglobulin precursor [KO:K03910]
24231  C2; complement C2 precursor [KO:K01332] [EC:3.4.21.43]
24232  C3; complement C3 precursor [KO:K03990]
24233  C4a; complement C4 precursor [KO:K03989]
24235  C4bpa; C4b-binding protein alpha chain [KO:K04002]
24236  C4bpb; C4b-binding protein beta chain precursor [KO:K04003]
24237  C6; complement component C6 precursor [KO:K03995]
24366  Fgb; fibrinogen beta chain precursor [KO:K03904]
24367  Fgg; fibrinogen gamma chain precursor [KO:K03905]
24548  Mbl1; mannose-binding protein A precursor [KO:K03991]
24617  Serpine1; plasminogen activator inhibitor 1 precursor [KO:K03982]
24648  Serpina1; alpha-1-antiproteinase precursor [KO:K03984]
24854  Clu; clusterin precursor [KO:K17252]
24903  Kng2; T-kininogen 1 precursor [KO:K03898]
24946  F9; coagulation factor IX precursor [KO:K01321] [EC:3.4.21.22]
25021  Itgam; integrin alpha-M precursor [KO:K06461]
25048  Klkb1; plasma kallikrein precursor [KO:K01324] [EC:3.4.21.34]
25087  Kng2l1; kininogen-1 isoform 1 precursor [KO:K03898]
25245  Bdkrb2; B2 bradykinin receptor isoform 2 [KO:K03916]
25268  Proc; vitamin K-dependent protein C precursor [KO:K01344] [EC:3.4.21.69]
25407  Cd59b; CD59 glycoprotein precursor [KO:K04008]
25439  F2r; proteinase-activated receptor 1 precursor [KO:K03914]
25584  F3; tissue factor precursor [KO:K03901]
25619  Plau; urokinase-type plasminogen activator precursor [KO:K01348] [EC:3.4.21.73]
25692  Plat; tissue-type plasminogen activator precursor [KO:K01343] [EC:3.4.21.68]
260320  F7; coagulation factor VII precursor [KO:K01320] [EC:3.4.21.21]
287527  Serpinf2; alpha-2-antiplasmin precursor [KO:K03983]
288001  Kng1; T-kininogen 2 precursor [KO:K03898]
289055  F13b; coagulation factor XIII B chain precursor [KO:K03906]
289057  Cfhr1; complement factor H-related protein 1 precursor [KO:K23815]
289395  Cr2; complement receptor type 2 precursor [KO:K04012]
290757  F11; coagulation factor XI precursor [KO:K01323] [EC:3.4.21.27]
29169  Vtn; vitronectin precursor [KO:K06251]
29243  F10; coagulation factor X precursor [KO:K01314] [EC:3.4.21.6]
29251  F2; prothrombin precursor [KO:K01313] [EC:3.4.21.5]
29333  Cd46; membrane cofactor protein precursor [KO:K04007]
29366  Serpine2; glia-derived nexin precursor [KO:K16643]
294257  Cfb; complement factor B precursor [KO:K01335] [EC:3.4.21.47]
29436  Tfpi; tissue factor pathway inhibitor isoform 1 precursor [KO:K03909]
295703  Serping1; plasma protease C1 inhibitor precursor [KO:K04001]
29636  F2rl2; proteinase-activated receptor 3 precursor [KO:K04235]
296545  C8g; complement component C8 gamma chain isoform 1 precursor [KO:K03999]
29687  C1qb; complement C1q subcomponent subunit B precursor [KO:K03987]
298288  C8a; complement component C8 alpha chain precursor [KO:K03997]
298566  C1qa; complement C1q subcomponent subunit A precursor [KO:K03986]
302470  F8; coagulation factor VIII precursor [KO:K03899]
304917  Serpinc1; antithrombin-III precursor [KO:K03911]
304929  F5; coagulation factor V precursor [KO:K03902]
306761  F12; coagulation factor XII precursor [KO:K01328] [EC:3.4.21.38]
309684  Itgb2; integrin beta-2 precursor [KO:K06464]
312102  Vsig4; V-set and immunoglobulin domain-containing protein 4 precursor [KO:K19822]
312705  C1r; complement C1r subcomponent precursor [KO:K01330] [EC:3.4.21.41]
313421  C8b; complement component C8 beta chain precursor [KO:K03998]
361969  Fga; fibrinogen alpha chain isoform 1 precursor [KO:K03903]
362119  C5; complement C5 precursor [KO:K03994]
362248  Procr; endothelial protein C receptor precursor [KO:K06557]
362634  C1qc; complement C1q subcomponent subunit C precursor [KO:K03988]
365577  Cd46l1; membrane cofactor protein-like [KO:K04007]
406161  C4b; complement component 4, gene 2 precursor [KO:K03989]
499271  Itgax; integrin alpha-X precursor [KO:K06462]
50692  Plaur; urokinase plasminogen activator surface receptor precursor [KO:K03985]
54243  Cr1l; complement component receptor 1-like protein isoform 2 precursor [KO:K04011]
54249  Cfd; complement factor D precursor [KO:K01334] [EC:3.4.21.46]
60325  Serpinb2; plasminogen activator inhibitor 2 type A [KO:K19821]
60327  F13a1; coagulation factor XIII A chain [KO:K03917] [EC:2.3.2.13]
64023  Masp1; mannan-binding lectin serine protease 1 precursor [KO:K03992] [EC:3.4.21.-]
64036  Cd55; complement decay-accelerating factor precursor [KO:K04006]
64459  Masp2; mannan-binding lectin serine protease 2 precursor [KO:K03993] [EC:3.4.21.104]
64668  Mbl2; mannose-binding protein C precursor [KO:K03991]
65051  Serpina5; plasma serine protease inhibitor isoform 1 [KO:K03913]
688887  Cfhr2; complement factor H-related 2 precursor [KO:K23816]
79126  Cfi; complement factor I precursor [KO:K01333] [EC:3.4.21.45]
79224  Serpind1; heparin cofactor 2 precursor [KO:K03912]
81509  Bdkrb1; B1 bradykinin receptor [KO:K03915]
81750  Pros1; vitamin K-dependent protein S precursor [KO:K03908]
83580  Thbd; thrombomodulin precursor [KO:K03907]
84007  C3ar1; C3a anaphylatoxin chemotactic receptor [KO:K04009]
85253  Plg; plasminogen precursor [KO:K01315] [EC:3.4.21.7]
Compound
C00290  Fibrin
C00306  Bradykinin
C00471  Plasmin
C00752  Thrombin
C01065  Coagulation factor Xa
C01715  Plasma kallikrein
C02002  C3a anaphylatoxin
C03259  Coagulation factor IXa
C03262  Coagulation factor XIa
C03378  Coagulation factor VIIa
C03379  Coagulation factor XIIa
C03780  Coagulation factor Va
C04001  Coagulation factor VIIIa
C16510  C5a anaphylatoxin
C22531  Complement component 2a
C22532  Complement component 2b
C22533  Complement component 3b
C22534  Complement component 3f
C22535  Complement iC3b
C22536  Complement component 3dg
C22537  Complement component 3c
C22538  Complement component 3d
C22539  Complement C3(H2O)
C22540  Complement factor Bb
C22541  Complement component 4a
C22542  Complement component 4b
C22543  Complement component 4c
C22544  Complement component 4d
C22545  Complement component 5b
C22546  Coagulation factor XIIIa
C22547  APC
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)
DOI:10.15252/embj.201591881
Reference
  Authors
Mathern DR, Heeger PS
  Title
Molecules Great and Small: The Complement System.
  Journal
Clin J Am Soc Nephrol 10:1636-50 (2015)
DOI:10.2215/CJN.06230614
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)
DOI:10.3389/fimmu.2015.00262
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)
DOI:10.1007/s00281-011-0280-x
Reference
  Authors
Kurosawa S, Stearns-Kurosawa DJ
  Title
Complement, thrombotic microangiopathy and disseminated intravascular coagulation.
  Journal
J Intensive Care 2:65 (2014)
DOI:10.1186/s40560-014-0061-4
Reference
  Authors
Hillmeister P, Persson PB
  Title
The Kallikrein-Kinin system.
  Journal
Acta Physiol (Oxf) 206:215-9 (2012)
DOI:10.1111/apha.12007
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)
DOI:10.1111/j.1538-7836.2007.02516.x
Reference
  Authors
Crooks MG, Hart SP
  Title
Coagulation and anticoagulation in idiopathic pulmonary fibrosis.
  Journal
Eur Respir Rev 24:392-9 (2015)
DOI:10.1183/16000617.00008414
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)
DOI:10.1152/ajplung.00093.2013
Reference
  Authors
Alberelli MA, De Candia E
  Title
Functional role of protease activated receptors in vascular biology.
  Journal
Vascul Pharmacol 62:72-81 (2014)
DOI:10.1016/j.vph.2014.06.001
Reference
  Authors
Smith HW, Marshall CJ
  Title
Regulation of cell signalling by uPAR.
  Journal
Nat Rev Mol Cell Biol 11:23-36 (2010)
DOI:10.1038/nrm2821
Reference
  Authors
Ricklin D, Lambris JD
  Title
Complement in immune and inflammatory disorders: pathophysiological mechanisms.
  Journal
J Immunol 190:3831-8 (2013)
DOI:10.4049/jimmunol.1203487
Reference
  Authors
Ricklin D, Lambris JD
  Title
Complement in immune and inflammatory disorders: therapeutic interventions.
  Journal
J Immunol 190:3839-47 (2013)
DOI:10.4049/jimmunol.1203200
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)
DOI:10.3389/fcimb.2014.00128
Reference
  Authors
Jozsi M, Tortajada A, Uzonyi B, Goicoechea de Jorge E, Rodriguez de Cordoba S
  Title
Factor H-related proteins determine complement-activating surfaces.
  Journal
Trends Immunol 36:374-84 (2015)
DOI:10.1016/j.it.2015.04.008
Related
pathway
rno04611  Platelet activation
rno04662  B cell receptor signaling pathway
rno04750  Inflammatory mediator regulation of TRP channels
KO pathway
ko04610   

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