BioPAX pathway converted from "factor V -> factor Va + factor V activation peptide" in the Reactome database. 3.4.21.1 3.4.21.92 3.4.21.73 3.4.21.71 3.4.21.93 3.4.21.94 3.4.21.34 3.4.21.78 3.4.21.9 3.4.21.53 3.4.21.6 3.4.21.75 3.4.24.3 3.4.21.10 3.4.21.54 3.4.21.7 3.4.21.4 3.4.21.59 3.4.21.38 3.4.21.5 3.4.21.35 3.4.21.79 3.4.21.36 3.4.19.1 3.4.21.62 3.4.21.41 3.4.21.61 3.4.21.83 3.4.21.22 3.4.21.88 3.4.21.45 3.4.21.89 3.4.21.20 3.4.21.42 3.4.21.21 3.4.21.43 3.4.21.87 3.4.21.26 3.4.21.48 3.4.24.34 3.4.21.27 3.4.21.46 3.4.21.68 3.4.21.47 3.4.21.69 3.4.21.39 3.4.24.7 3.4.21.102 factor V -> factor Va + factor V activation peptide factor V -> factor Va + factor V activation peptide Activated thrombin (factor IIa) catalyzes the conversion of factor V to factor Va (activated factor V). The activation peptide released in this reaction has no known function. Authored: D'Eustachio, P, 2004-08-24 14:00:00 Reactome DB_ID: 140697 1 extracellular region GO 0005576 UniProt:P12259 F5 F5 F5 FUNCTION Central regulator of hemostasis. It serves as a critical cofactor for the prothrombinase activity of factor Xa that results in the activation of prothrombin to thrombin.ACTIVITY REGULATION Inhibited by SERPINA5.SUBUNIT Factor Va, the activated form of factor V, is composed of a heavy chain and a light chain, non-covalently bound. The interaction between the two chains is calcium-dependent. Forms heterodimer with SERPINA5.TISSUE SPECIFICITY Plasma.DOMAIN Domain B contains 35 x 9 AA tandem repeats, and 2 x 17 AA repeats.PTM Thrombin activates factor V proteolytically to the active cofactor, factor Va (formation of a heavy chain at the N-terminus and a light chain at the C-terminus).PTM Sulfation is required for efficient thrombin cleavage and activation and for full procoagulant activity.PTM Activated protein C inactivates factor V and factor Va by proteolytic degradation.PTM Phosphorylated by FAM20C in the extracellular medium.SIMILARITY Belongs to the multicopper oxidase family. Reactome http://www.reactome.org Homo sapiens NCBI Taxonomy 9606 UniProt P12259 Chain Coordinates 29 EQUAL 2224 EQUAL Reactome DB_ID: 140787 1 738 EQUAL 1573 EQUAL Reactome DB_ID: 140692 1 factor Va [extracellular region] factor Va Reactome DB_ID: 140691 1 29 EQUAL 737 EQUAL Reactome DB_ID: 140690 1 1574 EQUAL 2224 EQUAL Reactome DB_ID: 74112 1 calcium(2+) [ChEBI:29108] calcium(2+) ChEBI 29108 Reactome Database ID Release 83 140692 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=140692 Reactome R-HSA-140692 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-140692.1 PHYSIOL-LEFT-TO-RIGHT ACTIVATION Reactome DB_ID: 156786 activated thrombin (factor IIa) [extracellular region] activated thrombin (factor IIa) Reactome DB_ID: 140598 1 UniProt:P00734 F2 F2 F2 FUNCTION Thrombin, which cleaves bonds after Arg and Lys, converts fibrinogen to fibrin and activates factors V, VII, VIII, XIII, and, in complex with thrombomodulin, protein C. Functions in blood homeostasis, inflammation and wound healing.ACTIVITY REGULATION Inhibited by SERPINA5.SUBUNIT Heterodimer (named alpha-thrombin) of a light and a heavy chain; disulfide-linked. Forms a heterodimer with SERPINA5. In plasma, interacts (via N-terminus) with alpha-1-microglobulin with molar ratio 1:2 and 1:1; this interaction does not prevent the activation of prothrombin to thrombin.TISSUE SPECIFICITY Expressed by the liver and secreted in plasma.PTM The gamma-carboxyglutamyl residues, which bind calcium ions, result from the carboxylation of glutamyl residues by a microsomal enzyme, the vitamin K-dependent carboxylase. The modified residues are necessary for the calcium-dependent interaction with a negatively charged phospholipid surface, which is essential for the conversion of prothrombin to thrombin.PTM N-glycosylated. N-glycan heterogeneity at Asn-121: Hex3HexNAc3 (minor), Hex4HexNAc3 (minor) and Hex5HexNAc4 (major). At Asn-143: Hex4HexNAc3 (minor) and Hex5HexNAc4 (major).PHARMACEUTICAL The peptide TP508 also known as Chrysalin (Orthologic) could be used to accelerate repair of both soft and hard tissues.MISCELLANEOUS Prothrombin is activated on the surface of a phospholipid membrane that binds the amino end of prothrombin and factors Va and Xa in Ca-dependent interactions; factor Xa removes the activation peptide and cleaves the remaining part into light and heavy chains. The activation process starts slowly because factor V itself has to be activated by the initial, small amounts of thrombin.MISCELLANEOUS It is not known whether 1 or 2 smaller activation peptides, with additional cleavage after Arg-314, are released in natural blood clotting.MISCELLANEOUS Thrombin can itself cleave the N-terminal fragment (fragment 1) of the prothrombin, prior to its activation by factor Xa.MISCELLANEOUS The cleavage after Arg-198, observed in vitro, does not occur in plasma.SIMILARITY Belongs to the peptidase S1 family. UniProt P00734 328 EQUAL 363 EQUAL Reactome DB_ID: 140589 1 364 EQUAL 622 EQUAL Reactome DB_ID: 74112 6 Reactome Database ID Release 83 156786 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=156786 Reactome R-HSA-156786 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-156786.1 GO 0004252 GO molecular function Reactome Database ID Release 83 139890 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=139890 Reactome Database ID Release 83 140696 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=140696 Reactome R-HSA-140696 3 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-140696.3 14982929 Pubmed 2004 The factor V activation paradox Orfeo, T Brufatto, N Nesheim, ME Xu, H Butenas, S Mann, KG J Biol Chem 279:19580-91 INHIBITION Reactome Database ID Release 83 5607785 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=5607785 Reactome R-HSA-5607785 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-5607785.1 Reactome DB_ID: 5607781 plasma membrane GO 0005886 SERPINE2:GAG:activated thrombin (factor IIa) [plasma membrane] SERPINE2:GAG:activated thrombin (factor IIa) Reactome DB_ID: 140811 1 activated thrombin (factor IIa) [plasma membrane] activated thrombin (factor IIa) Reactome DB_ID: 140809 1 364 EQUAL 622 EQUAL Reactome DB_ID: 140648 6 Reactome DB_ID: 140810 1 328 EQUAL 363 EQUAL Reactome Database ID Release 83 140811 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=140811 Reactome R-HSA-140811 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-140811.1 Reactome DB_ID: 5607782 1 SERPINE2:GAG [plasma membrane] SERPINE2:GAG Reactome DB_ID: 4127459 2 UniProt:P07093 SERPINE2 SERPINE2 PN1 SERPINE2 PI7 FUNCTION Serine protease inhibitor with activity toward thrombin, trypsin, and urokinase. Promotes neurite extension by inhibiting thrombin. Binds heparin.SIMILARITY Belongs to the serpin family. UniProt P07093 20 EQUAL 398 EQUAL Reactome DB_ID: 977180 1 glycosaminoglycan [ChEBI:18085] glycosaminoglycan ChEBI 18085 Reactome Database ID Release 83 5607782 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=5607782 Reactome R-HSA-5607782 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-5607782.1 Reactome Database ID Release 83 5607781 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=5607781 Reactome R-HSA-5607781 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-5607781.1 INHIBITION Reactome Database ID Release 83 5607095 Database identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser?DB=gk_current&ID=5607095 Reactome R-HSA-5607095 1 Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: http://www.reactome.org/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-5607095.1 Converted from EntitySet in Reactome Reactome DB_ID: 5607050 Extracellular thrombin inactivating complexes [extracellular region] Converted from EntitySet in Reactome. Each synonym is a name of a PhysicalEntity, and each XREF points to one PhysicalEntity