KEGG   PATHWAY: oas04610
Entry
oas04610                    Pathway                                
Name
Complement and coagulation cascades - Ovis aries (sheep)
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
oas04610  Complement and coagulation cascades
oas04610

Organism
Ovis aries (sheep) [GN:oas]
Gene
100302501  PLAU; urokinase-type plasminogen activator precursor [KO:K01348] [EC:3.4.21.73]
100302564  PLAUR; urokinase plasminogen activator surface receptor precursor [KO:K03985]
100307041  F2; prothrombin precursor [KO:K01313] [EC:3.4.21.5]
100359363  F8; coagulation factor VIII precursor [KO:K03899]
100381267  SERPINE1; plasminogen activator inhibitor 1 precursor [KO:K03982]
101102090  FGA; fibrinogen alpha chain [KO:K03903]
101102126  C1QA; complement C1q subcomponent subunit A isoform X1 [KO:K03986]
101102273  C8A; complement component C8 alpha chain isoform X1 [KO:K03997]
101102336  FGB; fibrinogen beta chain [KO:K03904]
101102522  C8B; complement component C8 beta chain [KO:K03998]
101102670  CD59; CD59 glycoprotein [KO:K04008]
101102674  C6; complement component C6 [KO:K03995]
101102931  C7; complement component C7 [KO:K03996]
101103256  THBD; thrombomodulin [KO:K03907]
101104226  C3AR1; C3a anaphylatoxin chemotactic receptor [KO:K04009]
101104705  SERPINB2; plasminogen activator inhibitor 2 [KO:K19821]
101105026  C9; complement component C9 [KO:K04000]
101105044  [KO:K19821]
101105757  F2R; proteinase-activated receptor 1 isoform X1 [KO:K03914]
101105935  [KO:K04007]
101106447  [KO:K23815]
101106739  ITGAX; integrin alpha-X precursor [KO:K06462]
101106770  F12; coagulation factor XII isoform X1 [KO:K01328] [EC:3.4.21.38]
101107430  C1R; complement C1r subcomponent isoform X1 [KO:K01330] [EC:3.4.21.41]
101107498  MBLA; LOW QUALITY PROTEIN: mannose-binding protein A [KO:K03991]
101107688  C1S; complement C1s subcomponent [KO:K01331] [EC:3.4.21.42]
101108131  C3; complement C3 [KO:K03990]
101108612  F13A1; coagulation factor XIII A chain [KO:K03917] [EC:2.3.2.13]
101110677  F2RL2; proteinase-activated receptor 3 [KO:K04235]
101110931  VWF; von Willebrand factor [KO:K03900]
101111065  MASP1; mannan-binding lectin serine protease 1 isoform X1 [KO:K03992] [EC:3.4.21.-]
101112066  PLG; plasminogen [KO:K01315] [EC:3.4.21.7]
101112329  CPB2; carboxypeptidase B2 isoform X1 [KO:K01300] [EC:3.4.17.20]
101112781  KNG1; kininogen-1 isoform X1 [KO:K03898]
101112891  [KO:K03989]
101113203  VSIG4; V-set and immunoglobulin domain-containing protein 4 isoform X2 [KO:K19822]
101113728  [KO:K17252]
101114204  C4BPB; C4b-binding protein beta chain precursor [KO:K04003]
101114456  [KO:K04002]
101114699  F2RL3; proteinase-activated receptor 4 [KO:K04236]
101114941  [KO:K23816]
101115072  CFB; complement factor B isoform X2 [KO:K01335] [EC:3.4.21.47]
101115321  C2; complement C2 isoform X3 [KO:K01332] [EC:3.4.21.43]
101115705  CFD; complement factor D [KO:K01334] [EC:3.4.21.46]
101115814  PROCR; endothelial protein C receptor isoform X3 [KO:K06557]
101116362  MBL2; mannose-binding protein C [KO:K03991]
101116376  [KO:K01324] [EC:3.4.21.34]
101116630  [KO:K01323] [EC:3.4.21.27]
101117258  SERPINF2; alpha-2-antiplasmin isoform X2 [KO:K03983]
101118296  C5AR1; C5a anaphylatoxin chemotactic receptor 1 [KO:K04010]
101118332  PLAT; tissue-type plasminogen activator [KO:K01343] [EC:3.4.21.68]
101118369  F7; coagulation factor VII isoform X2 [KO:K01320] [EC:3.4.21.21]
101118510  [KO:K17252]
101118624  F10; coagulation factor X [KO:K01314] [EC:3.4.21.6]
101118918  F3; tissue factor [KO:K03901]
101119231  VTN; vitronectin [KO:K06251]
101119257  SERPINA5; plasma serine protease inhibitor isoform X2 [KO:K03913]
101119307  CFI; complement factor I isoform X2 [KO:K01333] [EC:3.4.21.45]
101119740  C4BPA; C4b-binding protein alpha chain isoform X3 [KO:K04002]
101120257  CD55; complement decay-accelerating factor isoform X14 [KO:K04006]
101120391  TFPI; tissue factor pathway inhibitor [KO:K03909]
101120781  BDKRB2; B2 bradykinin receptor [KO:K03916]
101121454  SERPING1; plasma protease C1 inhibitor [KO:K04001]
101121825  C5; complement C5 [KO:K03994]
101122004  SERPINE2; glia-derived nexin [KO:K16643]
101122294  CD46; membrane cofactor protein isoform X1 [KO:K04007]
101122712  F13B; coagulation factor XIII B chain isoform X4 [KO:K03906]
101122940  A2M; alpha-2-macroglobulin isoform X4 [KO:K03910]
101122966  CFHR5; complement factor H-related protein 5 isoform X1 [KO:K23817]
101122999  PROS1; vitamin K-dependent protein S [KO:K03908]
101123189  C1QC; complement C1q subcomponent subunit C isoform X1 [KO:K03988]
101123223  [KO:K23815]
101123419  [KO:K03989]
101123649  F5; coagulation factor V [KO:K03902]
101123664  FGG; fibrinogen gamma chain isoform X2 [KO:K03905]
101123672  [KO:K03989]
105601889  PROC; LOW QUALITY PROTEIN: vitamin K-dependent protein C [KO:K01344] [EC:3.4.21.69]
105602977  SERPIND1; heparin cofactor 2 [KO:K03912]
105611355  [KO:K04007]
105611356  [KO:K04007]
105614152  [KO:K04011]
106990096  [KO:K04004]
106991729  BDKRB1; B1 bradykinin receptor [KO:K03915]
114113848  C8G; complement component C8 gamma chain [KO:K03999]
114114921  [KO:K03990]
121818782  C1QB; complement C1q subcomponent subunit B [KO:K03987]
443017  CR2; complement receptor type 2 [KO:K04012]
443217  ITGAM; integrin alpha-M precursor [KO:K06461]
443316  F9; coagulation factor IX [KO:K01321] [EC:3.4.21.22]
443388  SERPINA1; alpha-1-antiproteinase precursor [KO:K03984]
443407  SERPINC1; antithrombin-III precursor [KO:K03911]
494433  ITGB2; integrin beta-2 precursor [KO:K06464]
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
oas04611  Platelet activation
oas04662  B cell receptor signaling pathway
oas04750  Inflammatory mediator regulation of TRP channels
KO pathway
ko04610   

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