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Factor IX

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424: 401: 3709: 298: 323: 3754: 3769: 3814: 3784: 3724: 3799: 3829: 3739: 430: 329: 675: 1263: 5099: 1398: 31: 5111: 1363: 1968:). Over 3000 variants of factor IX have been described, affecting 73% of the 461 residues; some cause no symptoms, but many lead to a significant bleeding disorder. The original Christmas disease mutation was identified by sequencing of Christmas' DNA, revealing a mutation which changed a cysteine to a serine. 57: 1381:
The structures of all four domains have been solved. A structure of the two EGF domains and the trypsin-like domain was determined for the pig protein. The structure of the Gla domain, which is responsible for Ca(II)-dependent phospholipid binding, was also determined by
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Taylor SA, Duffin J, Cameron C, Teitel J, Garvey B, Lillicrap DP (Jan 1992). "Characterization of the original Christmas disease mutation (cysteine 206----serine): from clinical recognition to molecular pathogenesis".
1421:: males experience the disease phenotype much more frequently than females. At least 534 disease-causing mutations in this gene have been discovered. The F9 gene was first cloned in 1982 by Kotoku Kurachi and 3753: 1952: 1866: 1748: 1554: 3768: 5171: 3813: 4753: 1916: 1830: 1712: 1609: 1518: 3783: 3723: 2719: 4613: 3245: 3215: 3185: 3155: 3125: 3095: 3065: 3035: 437: 336: 4353: 3869: 1324:
Factor IX expression increases with age in humans and mice. In mouse models, mutations within the promoter region of factor IX have an age-dependent phenotype.
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Several structures of 'super active' mutants have been solved, which reveal the nature of factor IX activation by other proteins in the clotting cascade.
3828: 938: 2776: 919: 259: 2341:"The N-terminal epidermal growth factor-like domain in factor IX and factor X represents an important recognition motif for binding to tissue factor" 5136: 3738: 3532: 2480:
Freedman SJ, Furie BC, Furie B, Baleja JD (Sep 1995). "Structure of the calcium ion-bound gamma-carboxyglutamic acid-rich domain of factor IX".
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may be of value in patients undergoing surgery who have inherited factor IX deficiency in order to reduce the perioperative risk of bleeding.
5151: 3688: 2115: 2035: 1251: 2097: 5131: 4130: 1378:. conclude that residues 88 to 109 of the second EGF domain mediate binding to platelets and assembly of the factor X activating complex. 4346: 2972: 2939: 3005: 2906: 2382:"The factor IXa second epidermal growth factor (EGF2) domain mediates platelet binding and assembly of the factor X activating complex" 1154: 423: 5156: 3862: 3717:: STRUCTURE OF THE METAL-FREE GAMMA-CARBOXYGLUTAMIC ACID-RICH MEMBRANE BINDING REGION OF FACTOR IX BY TWO-DIMENSIONAL NMR SPECTROSCOPY 3657: 2024:
Factor IX deficiency is treated by injection of purified factor IX produced through cloning in various animal or animal cell vectors.
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Lenting PJ, van Mourik JA, Mertens K (Dec 1998). "The life cycle of coagulation factor VIII in view of its structure and function".
1161: 4307: 400: 1819: 1701: 1507: 3237: 3207: 3177: 3147: 3117: 3087: 3057: 3027: 4135: 3971: 2084: 2063: 1937: 1851: 1800: 1733: 1682: 1630: 1539: 1488: 1452: 4339: 3911: 4974: 2080: 322: 297: 54: 4211: 3855: 5089: 2558:"Refinement of evolutionary medicine predictions based on clinical evidence for the manifestations of Mendelian diseases" 2059: 4573: 4163: 239: 5141: 4331: 3267:
Simioni P, Tormene D, Tognin G, Gavasso S, Bulato C, Iacobelli NP, Finn JD, Spiezia L, Radu C, Arruda VR (Oct 2009).
2423:"X-ray structure of clotting factor IXa: active site and module structure related to Xase activity and hemophilia B" 2013:
Some rare mutations of factor IX result in elevated clotting activity, and can result in clotting diseases, such as
4959: 436: 335: 5075: 5062: 5049: 5036: 5023: 5010: 4997: 4488: 4473: 4373: 4251: 3681: 2999: 2966: 2933: 2900: 2752: 2695: 2188: 4969: 3762:: THE THREE-DIMENSIONAL STRUCTURE OF THE FIRST EGF-LIKE MODULE OF HUMAN FACTOR IX: COMPARISON WITH EGF AND TGF-A 4923: 4866: 4370: 1217: 983: 429: 328: 247: 4871: 4763: 4728: 4723: 1595: 964: 4077: 4073: 230:, F9 p22, FIX, HEMB, P19, PTC, THPH8, coagulation factor IX, Blood coagulation factor IX, Christmas Factor 4892: 4811: 4718: 4568: 4192: 3936: 3905: 2021:
mutation renders the protein hyperfunctional and is associated with familial early-onset thrombophilia.
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Human factor IX protein domain architecture, where each protein domain is represented by a coloured box
3363: 4708: 4233: 3777:: Crystal Structure of Mg(II)-and Ca(II)-bound Gla Domain of Factor IX Complexed with Binding Protein 3674: 3491: 2626: 2569: 2517:"Structural basis of the cofactor- and substrate-assisted activation of human coagulation factor IXa" 2434: 2014: 311: 226: 4928: 4608: 4598: 4415: 4410: 4243: 4220: 4201: 4183: 3878: 1133: 1112: 1082: 1057: 5146: 4861: 3886: 3604: 3550:
O'Connell NM (Jun 2003). "Factor XI deficiency--from molecular genetics to clinical management".
3524: 3449: 2877: 2313: 2296: 1418: 271: 1137: 1108: 1078: 1053: 3420:"Assessing the underlying pattern of human germline mutations: lessons from the factor IX gene" 1302:, in the presence of Ca, membrane phospholipids, and a Factor VIII cofactor, it hydrolyses one 5161: 4590: 4271: 4228: 3624: 3596: 3567: 3516: 3478: 3441: 3406: 3341: 3300: 2869: 2833: 2654: 2595: 2538: 2497: 2462: 2403: 2362: 2318: 2277: 2250: 2167: 1906: 1790: 1760: 1672: 1585: 1478: 1340: 1243: 219: 47: 3822:: Crystal structure of human factor IX Gla domain in complex of an inhibitory antibody, 10C12 2961: 2928: 2747: 2297:"Age-specific regulation of clotting factor IX gene expression in normal and transgenic mice" 1294:(of the tissue factor pathway) to produce a two-chain form, where the chains are linked by a 4907: 4902: 4876: 4804: 4382: 4284: 4279: 3588: 3559: 3506: 3470: 3431: 3398: 3331: 3290: 3280: 2994: 2895: 2861: 2823: 2815: 2690: 2644: 2634: 2585: 2577: 2528: 2489: 2452: 2442: 2393: 2352: 2308: 2240: 2232: 2196: 2157: 2149: 1440: 1295: 516: 447: 391: 346: 3654: 5166: 5115: 4954: 4938: 4851: 4623: 4618: 4390: 4366: 3951: 3930: 3916: 3792:: Crystal Structure of Ca(II)-bound Gla Domain of Factor IX Complexed with Binding Protein 3661: 3579:
Du X (May 2007). "Signaling and regulation of the platelet glycoprotein Ib-IX-V complex".
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Boland EJ, Liu YC, Walter CA, Herbert DC, Weaker FJ, Odom MW, Jagadeeswaran P (Sep 1995).
2025: 1969: 1428: 1352: 1228: 491: 3732:: NMR STRUCTURE OF CALCIUM ION-BOUND GAMMA-CARBOXY-GLUTAMIC ACID-RICH DOMAIN OF FACTOR IX 3402: 2630: 2573: 2438: 1370:
The N-terminal EGF domain has been shown to at least in part be responsible for binding
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and also share a common domain architecture. The factor IX protein is composed of four
1279: 674: 3320:"Do patients with haemophilia undergoing cardiac surgery have good surgical outcomes?" 2649: 2614: 2245: 2220: 853: 848: 843: 838: 833: 828: 823: 818: 813: 797: 792: 787: 782: 777: 772: 767: 751: 746: 741: 736: 731: 726: 721: 716: 5125: 4897: 4856: 4768: 4641: 4630: 4362: 4102: 4026: 3981: 3633: 3563: 3511: 2724: 2457: 2422: 1371: 1283: 1236: 703: 3608: 3528: 3453: 2881: 4846: 4664: 4558: 4400: 4299: 4145: 4110: 1965: 1414: 1402: 1318: 509: 288: 251: 3592: 275: 5070: 5005: 4841: 4783: 4778: 4427: 4153: 4088: 4059: 4041: 4016: 4006: 3961: 3895: 3436: 3419: 2120:
National Center for Biotechnology Information, U.S. National Library of Medicine
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National Center for Biotechnology Information, U.S. National Library of Medicine
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Proceedings of the National Academy of Sciences of the United States of America
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Proceedings of the National Academy of Sciences of the United States of America
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A list of all the mutations in Factor IX is compiled and maintained by EAHAD.
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Taran LD (Jul 1997). "Factor IX of the blood coagulation system: a review".
2153: 1287: 1267: 883: 652: 530: 475: 462: 374: 361: 263: 3600: 3571: 3520: 3345: 3304: 2865: 2837: 2599: 2542: 2407: 2398: 2381: 2366: 2357: 2340: 2193:
World Health Organization model list of essential medicines: 21st list 2019
2171: 3482: 3445: 3285: 3268: 2873: 2658: 2501: 2466: 2322: 2281: 1201: 30: 4659: 4654: 4603: 4453: 4438: 4405: 4064: 4054: 4049: 3410: 3295: 2254: 2018: 1814: 1696: 1502: 1336: 1311: 1303: 1220: 1185: 1028: 1009: 2493: 2201: 1196: 4713: 4703: 4649: 4563: 4395: 4316: 4158: 3638: 3628: 1356: 1275: 1235:; it belongs to peptidase family S1. Deficiency of this protein causes 995: 950: 3847: 3389:
Davie EW, Fujikawa K (1975). "Basic mechanisms in blood coagulation".
2819: 2236: 1362: 196: 192: 188: 184: 180: 176: 172: 168: 164: 160: 156: 152: 148: 144: 140: 136: 132: 128: 124: 120: 116: 112: 108: 104: 100: 96: 92: 88: 84: 80: 76: 5057: 4827: 4740: 4682: 4083: 3940: 3807:: COAGULATION FACTOR, MG(II), NMR, 7 STRUCTURES (BACKBONE ATOMS ONLY) 3650: 3088:"Alprolix (coagulation factor ix- recombinant, fc fusion protein kit" 2615:"Isolation and characterization of a cDNA coding for human factor IX" 1169: 905: 2138:"Christmas disease: a condition previously mistaken for haemophilia" 2136:
Biggs R, Douglas AS, Macfarlane RG, Dacie JV, Pitney WR (Dec 1952).
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factor IX is used to treat Christmas disease. Formulations include:
5031: 4749: 4735: 4674: 4548: 4543: 4538: 4533: 4528: 4523: 3925: 3920: 3837:: HUMAN COAGULATION FACTOR IXA IN COMPLEX WITH P-AMINO BENZAMIDINE 3269:"X-linked thrombophilia with a mutant factor IX (factor IX Padua)" 3178:"Rebinyn ((coagulation factor ix- recombinant, glycopegylated kit" 1396: 1361: 1261: 1997:
coagulation factor IX (recombinant), Fc fusion protein (Alprolix)
1359:-like peptidase domain which carries out the catalytic cleavage. 868: 864: 4518: 4513: 4508: 4503: 4498: 4493: 4483: 4478: 3945: 1873: 1639: 1410: 243: 4800: 4335: 3851: 3670: 2421:
Brandstetter H, Bauer M, Huber R, Lollar P, Bode W (Oct 1995).
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Protein Z-related protease inhibitor (ZPI) (inhibits FX, FXI)
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Overview of all the structural information available in the
2804:"Hemophilia B: Molecular pathogenesis and mutation analysis" 3359: 1946: 1860: 1742: 1548: 4796: 3747:: EPIDERMAL GROWTH FACTOR-LIKE DOMAIN FROM HUMAN FACTOR IX 1435:
sheep carrying the gene for factor IX, was produced by Dr
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endoplasmic reticulum to Golgi vesicle-mediated transport
2720:"Health Canada New Drug Authorizations: 2016 Highlights" 2205:. WHO/MVP/EMP/IAU/2019.06. License: CC BY-NC-SA 3.0 IGO. 1755: 1278:, an inactive precursor. It is processed to remove the 499: 3058:"Idelvion- coagulation factor ix recombinant human kit" 2036:
World Health Organization's List of Essential Medicines
1417:(Xq27.1-q27.2), loss-of-function mutations thereof are 1252:
World Health Organization's List of Essential Medicines
4136:
Tissue factor pathway inhibitor (TFPI) (inhibits FIII)
1246:
was found to be lacking this exact factor, leading to
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online database for peptidases and their inhibitors:
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An Introduction to Genetic Engineering Second Edition
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Zhong D, Bajaj MS, Schmidt AE, Bajaj SP (Feb 2002).
4983: 4947: 4916: 4885: 4834: 4696: 4673: 4639: 4589: 4426: 4381: 4298: 4270: 4242: 4219: 4210: 4144: 4101: 4040: 4015: 3960: 3894: 3885: 1936: 1931: 1915: 1905: 1900: 1850: 1845: 1829: 1799: 1789: 1784: 1732: 1727: 1711: 1681: 1671: 1666: 1629: 1624: 1608: 1594: 1584: 1579: 1538: 1533: 1517: 1487: 1477: 1472: 1126: 1101: 1071: 1046: 4202:Thrombin-activatable fibrinolysis inhibitor (TAFI) 2221:"Activation of human factor IX (Christmas factor)" 2076: 2074: 2072: 2055: 2053: 2051: 1964:Deficiency of factor IX causes Christmas disease ( 1242:It was discovered in 1952 after a young boy named 4262:Activated protein C–protein C inhibitor (APC–PCI) 3208:"Rixubis (coagulation factor ix- recombinant kit" 3148:"Ixinity (coagulation factor ix- recombinant kit" 3118:"Ixinity (coagulation factor ix- recombinant kit" 3028:"Benefix (coagulation factor ix- recombinant kit" 446: 345: 3238:"Alphanine SD (coagulation factor ix- human kit" 2777:"Refixia (Novo Nordisk Pharmaceuticals Pty Ltd)" 4111:Antithrombin (inhibits FII, FIX, FX, FXI, FXII) 3324:Interactive Cardiovascular and Thoracic Surgery 2685: 2683: 2334: 2332: 2219:Di Scipio RG, Kurachi K, Davie EW (Jun 1978). 2081:GRCm38: Ensembl release 89: ENSMUSG00000031138 5172:World Health Organization essential medicines 4812: 4347: 3863: 3682: 3645:GeneReviews/NCBI/NIH/UW entry on Hemophilia B 2380:Wilkinson FH, Ahmad SS, Walsh PN (Feb 2002). 2131: 2129: 8: 3360:"Home: EAHAD Factor 9 Gene Variant Database" 2314:10.1182/blood.V86.6.2198.bloodjournal8662198 2214: 2212: 2009:coagulation factor IX (human) (Alphanine SD) 1887: 1818: 1771: 1700: 1653: 1566: 1506: 1459: 2060:GRCh38: Ensembl release 89: ENSG00000101981 1982:albutrepenonacog alfa (brand name Idelvion) 4819: 4805: 4797: 4354: 4340: 4332: 4216: 3891: 3870: 3856: 3848: 3689: 3675: 3667: 2676:. Cambridge University Press. p. 257. 879: 699: 487: 386: 283: 65: 4179:Plasminogen activator inhibitor-2 (PAI-2) 4174:Plasminogen activator inhibitor-1 (PAI-1) 3510: 3435: 3335: 3318:Rossi M, Jayaram R, Sayeed R (Sep 2011). 3294: 3284: 2827: 2648: 2638: 2589: 2556:Ĺ imÄŤĂ­ková D, Heneberg P (December 2019). 2532: 2456: 2446: 2397: 2356: 2312: 2244: 2200: 2183: 2181: 2161: 1401:In human, the F9 gene is located on the 798:collagen-containing extracellular matrix 5094: 3704: 2047: 1988:nonacog beta pegol (brand name Refixia) 1985:eftrenonacog alfa (brand name Alprolix) 4367:serine proteases/serine endopeptidases 3972:High-molecular-weight kininogen (HMWK) 3128:from the original on 28 September 2023 2781:Therapeutic Goods Administration (TGA) 1886: 1770: 1652: 1565: 1458: 20: 3248:from the original on 18 February 2024 3188:from the original on 29 November 2022 2808:Journal of Thrombosis and Haemostasis 2225:The Journal of Clinical Investigation 2195:. Geneva: World Health Organization. 451: 412: 407: 350: 309: 304: 7: 3552:Blood Coagulation & Fibrinolysis 3158:from the original on 3 December 2022 3098:from the original on 7 February 2023 3068:from the original on 27 January 2023 3038:from the original on 29 January 2023 844:peptidyl-glutamic acid carboxylation 839:blood coagulation, extrinsic pathway 819:blood coagulation, intrinsic pathway 4257:Thrombin–antithrombin complex (TAT) 3403:10.1146/annurev.bi.44.070175.004055 3273:The New England Journal of Medicine 2975:from the original on 11 August 2020 2515:Zögg T, Brandstetter H (Dec 2009). 2386:The Journal of Biological Chemistry 2345:The Journal of Biological Chemistry 623:medial head of gastrocnemius muscle 4164:Tissue plasminogen activator (tPA) 4121:Protein S (cofactor for protein C) 1979:nonacog gamma (brand name Rixubis) 1250:. Coagulation factor IX is on the 1123: 1098: 1068: 1043: 1019: 1000: 974: 955: 929: 910: 747:serine-type endopeptidase activity 669: 587: 525: 504: 14: 3008:from the original on 18 June 2020 2942:from the original on 17 June 2020 2909:from the original on 17 June 2020 1994:coagulation factor IX (Idelvion) 1976:nonacog alfa (brand name Benefix) 5109: 5097: 3827: 3812: 3797: 3782: 3767: 3752: 3737: 3722: 3707: 3564:10.1097/00001721-200306001-00014 3512:10.1046/j.1365-2141.2001.03186.x 3218:from the original on 2 July 2022 2613:Kurachi K, Davie EW (Nov 1982). 2034:Coagulation factor IX is on the 2006:coagulation factor IX (Rixubis) 2003:coagulation factor IX (Rebinyn) 2000:coagulation factor IX (Ixinity) 1991:coagulation factor IX (Benefix) 1413:for factor IX is located on the 673: 435: 428: 422: 399: 334: 327: 321: 296: 29: 4252:Prothrombin fragment 1+2 (F1+2) 3912:Platelet membrane glycoproteins 3637:(Coagulation factor IX) at the 3538:from the original on 2021-06-19 3366:from the original on 2020-10-28 1453:Prothrombin complex concentrate 18:Protein involved in coagulation 5137:Drugs not assigned an ATC code 4116:Protein C (inhibits FV, FVIII) 3499:British Journal of Haematology 732:serine-type peptidase activity 684:More reference expression data 653:More reference expression data 1: 4579:Urinary plasminogen activator 3581:Current Opinion in Hematology 3391:Annual Review of Biochemistry 420: 319: 5152:Peripheral membrane proteins 4574:Tissue plasminogen activator 3593:10.1097/MOH.0b013e3280dce51a 1290:(of the contact pathway) or 569:human musculoskeletal system 5132:Genes on human chromosome X 3992:Factor XII (Hageman factor) 3906:von Willebrand factor (vWF) 3437:10.1096/fasebj.6.10.1634040 1351:, two tandem copies of the 1274:Factor IX is produced as a 768:endoplasmic reticulum lumen 631:nucleus of stria terminalis 5188: 4312:-antiplasmin complex (PAP) 4027:Factor III (tissue factor) 3492:"Factor IX and thrombosis" 2854:Thrombosis and Haemostasis 2582:10.1038/s41598-019-54976-4 1450: 1317:Factor IX is inhibited by 1266:The blood coagulation and 615:sexually immature organism 5157:Drugs developed by Pfizer 4975:Michaelis–Menten kinetics 4665:Proteinase 3/Myeloblastin 3702: 3475:10.1182/blood.V92.11.3983 3337:10.1510/icvts.2011.272401 3000:European Medicines Agency 2967:European Medicines Agency 2934:European Medicines Agency 2901:European Medicines Agency 2753:European Medicines Agency 2696:European Medicines Agency 2534:10.1016/j.str.2009.10.011 2189:World Health Organization 2116:"Mouse PubMed Reference:" 2098:"Human PubMed Reference:" 1200: 1195: 1191: 1184: 1168: 1155:Chr X: 139.53 – 139.56 Mb 1149: 1130: 1105: 1094: 1075: 1050: 1039: 1026: 1022: 1007: 1003: 994: 981: 977: 962: 958: 949: 936: 932: 917: 913: 904: 889: 882: 878: 862: 834:signal peptide processing 702: 698: 681: 672: 663: 650: 599: 590: 537: 528: 498: 490: 486: 469: 456: 419: 398: 389: 385: 368: 355: 318: 295: 286: 282: 237: 234: 224: 217: 212: 73: 68: 51: 46: 41: 37: 28: 23: 4867:Diffusion-limited enzyme 4234:β-Thromboglobulin (β-TG) 2270:Biochemistry. Biokhimiia 4229:Platelet factor 4 (PF4) 4126:Protein Z (inhibits FX) 4060:Prothrombin (factor II) 2640:10.1073/pnas.79.21.6461 2448:10.1073/pnas.92.21.9796 2154:10.1136/bmj.2.4799.1378 2142:British Medical Journal 1298:. When activated into 1162:Chr X: 59.04 – 59.08 Mb 4719:Proprotein convertases 4285:Fibrinopeptide B (FpB) 4280:Fibrinopeptide A (FpA) 3952:Glycoprotein VI (GPVI) 3917:Glycoprotein Ib (GPIb) 3418:Sommer SS (Jul 1992). 2866:10.1055/s-0038-1648381 2399:10.1074/jbc.M107753200 2358:10.1074/jbc.M111202200 1431:, a transgenic cloned 1406: 1367: 1339:all play key roles in 1271: 717:endopeptidase activity 607:lumbar spinal ganglion 553:pancreatic ductal cell 4960:Eadie–Hofstee diagram 4893:Allosteric regulation 4569:Plasminogen activator 4103:Anticoagulant factors 4070:Fibrinogen (factor I) 4065:Thrombin (factor IIa) 3937:Glycoprotein IIb/IIIa 3931:Glycoprotein IX (GP9) 3898:(platelet activation) 3286:10.1056/NEJMoa0904377 3004:. 17 September 2018. 2971:. 17 September 2018. 2938:. 17 September 2018. 2905:. 17 September 2018. 1820:Coagulation Factor IX 1702:Coagulation Factor IX 1662:albutrepenonacog alfa 1508:Coagulation Factor IX 1400: 1365: 1265: 783:extracellular exosome 4970:Lineweaver–Burk plot 4709:Prolyl endopeptidase 4290:Fibrin monomers (FM) 4146:Fibrinolytic factors 3964:(contact activation) 3490:Lowe GD (Dec 2001). 3124:. 23 February 2021. 2015:deep vein thrombosis 1286:and then cleaved by 788:extracellular region 414:X chromosome (mouse) 312:X chromosome (human) 69:List of PDB id codes 42:Available structures 4244:Thrombin generation 4221:Platelet activation 4212:Coagulation markers 3887:Coagulation factors 3879:Coagulation cascade 3558:(Suppl 1): S59-64. 3244:. 18 January 2024. 2802:Goodeve AC (2015). 2783:. 13 September 2024 2672:Nicholl D. (2002). 2631:1982PNAS...79.6461K 2574:2019NatSR...918577S 2494:10.1021/bi00038a005 2439:1995PNAS...92.9796B 1955:(Prescription only) 1897: 1869:(Prescription only) 1781: 1751:(Prescription only) 1663: 1576: 1557:(Prescription only) 1469: 1328:Domain architecture 1314:to form factor Xa. 793:extracellular space 742:calcium ion binding 541:right lobe of liver 453:X A6|X 33.5 cM 5142:Coagulation system 4929:Enzyme superfamily 4862:Enzyme promiscuity 4084:Fibrin (factor Ia) 3896:Primary hemostasis 3660:2005-05-05 at the 3184:. 11 August 2022. 3154:. 9 January 2024. 2700:. 19 December 2014 2562:Scientific Reports 1896:nonacog beta pegol 1419:X-linked recessive 1407: 1405:at position q27.1. 1368: 1272: 984:ENSMUSG00000031138 824:zymogen activation 807:Biological process 761:Cellular component 737:hydrolase activity 727:peptidase activity 710:Molecular function 619:thoracic diaphragm 603:left lobe of liver 5085: 5084: 4794: 4793: 4591:Complement system 4383:Digestive enzymes 4329: 4328: 4325: 4324: 4272:Fibrin generation 4097: 4096: 4017:Extrinsic pathway 3962:Intrinsic pathway 3845: 3844: 3214:. 22 March 2023. 2820:10.1111/jth.12958 2237:10.1172/JCI109073 2148:(4799): 1378–82. 1962: 1961: 1950: 1885: 1884: 1877: 1864: 1812: 1780:eftrenonacog alfa 1769: 1768: 1758: 1746: 1694: 1651: 1650: 1643: 1564: 1563: 1552: 1500: 1355:and a C-terminal 1341:blood coagulation 1244:Stephen Christmas 1223:), also known as 1211: 1210: 1207: 1206: 1180: 1179: 1145: 1144: 1120: 1119: 1090: 1089: 1065: 1064: 1035: 1034: 1016: 1015: 990: 989: 971: 970: 945: 944: 926: 925: 874: 873: 854:blood coagulation 722:metal ion binding 694: 693: 690: 689: 659: 658: 646: 645: 584: 583: 482: 481: 381: 380: 208: 207: 204: 203: 52:Ortholog search: 5179: 5114: 5113: 5112: 5102: 5101: 5093: 4965:Hanes–Woolf plot 4908:Enzyme activator 4903:Enzyme inhibitor 4877:Enzyme catalysis 4821: 4814: 4807: 4798: 4356: 4349: 4342: 4333: 4217: 3892: 3872: 3865: 3858: 3849: 3831: 3816: 3801: 3786: 3771: 3756: 3741: 3726: 3711: 3691: 3684: 3677: 3668: 3612: 3575: 3546: 3544: 3543: 3537: 3514: 3496: 3486: 3457: 3439: 3414: 3375: 3374: 3372: 3371: 3356: 3350: 3349: 3339: 3315: 3309: 3308: 3298: 3288: 3264: 3258: 3257: 3255: 3253: 3234: 3228: 3227: 3225: 3223: 3204: 3198: 3197: 3195: 3193: 3174: 3168: 3167: 3165: 3163: 3144: 3138: 3137: 3135: 3133: 3114: 3108: 3107: 3105: 3103: 3084: 3078: 3077: 3075: 3073: 3064:. 30 June 2023. 3054: 3048: 3047: 3045: 3043: 3034:. 1 March 2023. 3024: 3018: 3017: 3015: 3013: 2991: 2985: 2984: 2982: 2980: 2958: 2952: 2951: 2949: 2947: 2925: 2919: 2918: 2916: 2914: 2892: 2886: 2885: 2848: 2842: 2841: 2831: 2814:(7): 1184–1195. 2799: 2793: 2792: 2790: 2788: 2773: 2767: 2766: 2764: 2762: 2744: 2738: 2737: 2735: 2733: 2716: 2710: 2709: 2707: 2705: 2687: 2678: 2677: 2669: 2663: 2662: 2652: 2642: 2610: 2604: 2603: 2593: 2553: 2547: 2546: 2536: 2512: 2506: 2505: 2488:(38): 12126–37. 2477: 2471: 2470: 2460: 2450: 2433:(21): 9796–800. 2418: 2412: 2411: 2401: 2377: 2371: 2370: 2360: 2336: 2327: 2326: 2316: 2292: 2286: 2285: 2265: 2259: 2258: 2248: 2216: 2207: 2206: 2204: 2185: 2176: 2175: 2165: 2133: 2124: 2123: 2112: 2106: 2105: 2094: 2088: 2078: 2067: 2057: 2019:gain of function 1948: 1945: 1898: 1895: 1893: 1875: 1872: 1862: 1859: 1822: 1811: 1808: 1782: 1779: 1777: 1757: 1754: 1744: 1741: 1704: 1693: 1690: 1664: 1661: 1659: 1641: 1638: 1577: 1574: 1572: 1550: 1547: 1510: 1499: 1496: 1470: 1467: 1465: 1441:Roslin Institute 1296:disulfide bridge 1229:serine proteases 1227:, is one of the 1225:Christmas factor 1193: 1192: 1164: 1157: 1140: 1124: 1115: 1099: 1095:RefSeq (protein) 1085: 1069: 1060: 1044: 1020: 1001: 975: 956: 930: 911: 880: 700: 686: 677: 670: 655: 595: 593:Top expressed in 588: 545:secondary oocyte 533: 531:Top expressed in 526: 505: 488: 478: 465: 454: 439: 432: 426: 415: 403: 387: 377: 364: 353: 338: 331: 325: 314: 300: 284: 278: 229: 222: 199: 66: 60: 39: 38: 33: 21: 5187: 5186: 5182: 5181: 5180: 5178: 5177: 5176: 5122: 5121: 5120: 5110: 5108: 5096: 5088: 5086: 5081: 4993:Oxidoreductases 4979: 4955:Enzyme kinetics 4943: 4939:List of enzymes 4912: 4881: 4852:Catalytic triad 4830: 4825: 4795: 4790: 4692: 4669: 4635: 4585: 4422: 4391:Enteropeptidase 4377: 4360: 4330: 4321: 4311: 4294: 4266: 4238: 4206: 4196: 4187: 4140: 4093: 4036: 4019:(tissue factor) 4018: 4011: 3963: 3956: 3897: 3881: 3876: 3846: 3841: 3838: 3832: 3823: 3817: 3808: 3802: 3793: 3787: 3778: 3772: 3763: 3757: 3748: 3742: 3733: 3727: 3718: 3712: 3698: 3695: 3662:Wayback Machine 3620: 3615: 3578: 3549: 3541: 3539: 3535: 3494: 3489: 3469:(11): 3983–96. 3460: 3430:(10): 2767–74. 3417: 3388: 3384: 3382:Further reading 3379: 3378: 3369: 3367: 3358: 3357: 3353: 3317: 3316: 3312: 3266: 3265: 3261: 3251: 3249: 3236: 3235: 3231: 3221: 3219: 3206: 3205: 3201: 3191: 3189: 3176: 3175: 3171: 3161: 3159: 3146: 3145: 3141: 3131: 3129: 3116: 3115: 3111: 3101: 3099: 3094:. 25 May 2023. 3086: 3085: 3081: 3071: 3069: 3056: 3055: 3051: 3041: 3039: 3026: 3025: 3021: 3011: 3009: 2993: 2992: 2988: 2978: 2976: 2962:"Alprolix EPAR" 2960: 2959: 2955: 2945: 2943: 2929:"Idelvion EPAR" 2927: 2926: 2922: 2912: 2910: 2894: 2893: 2889: 2850: 2849: 2845: 2801: 2800: 2796: 2786: 2784: 2775: 2774: 2770: 2760: 2758: 2748:"Alprolix EPAR" 2746: 2745: 2741: 2731: 2729: 2728:. 14 March 2017 2718: 2717: 2713: 2703: 2701: 2689: 2688: 2681: 2671: 2670: 2666: 2612: 2611: 2607: 2555: 2554: 2550: 2527:(12): 1669–78. 2514: 2513: 2509: 2479: 2478: 2474: 2420: 2419: 2415: 2379: 2378: 2374: 2338: 2337: 2330: 2307:(6): 2198–205. 2294: 2293: 2289: 2267: 2266: 2262: 2218: 2217: 2210: 2187: 2186: 2179: 2135: 2134: 2127: 2114: 2113: 2109: 2096: 2095: 2091: 2079: 2070: 2058: 2049: 2044: 2026:Tranexamic acid 1958: 1927: 1890: 1889: 1881: 1841: 1825: 1774: 1773: 1765: 1723: 1707: 1656: 1655: 1647: 1620: 1597: 1569: 1568: 1560: 1529: 1513: 1462: 1461: 1455: 1449: 1447:Role in disease 1395: 1345:protein domains 1330: 1260: 1202:View/Edit Mouse 1197:View/Edit Human 1160: 1153: 1150:Location (UCSC) 1136: 1132: 1111: 1107: 1081: 1077: 1056: 1052: 965:ENSG00000101981 858: 802: 773:plasma membrane 756: 752:protein binding 682: 651: 642: 637: 635:piriform cortex 633: 629: 625: 621: 617: 613: 609: 605: 591: 580: 575: 573:muscular system 571: 567: 563: 559: 555: 551: 547: 543: 529: 473: 460: 452: 442: 441: 440: 433: 413: 390:Gene location ( 372: 359: 351: 341: 340: 339: 332: 310: 287:Gene location ( 238: 225: 218: 75: 53: 19: 12: 11: 5: 5185: 5183: 5175: 5174: 5169: 5164: 5159: 5154: 5149: 5144: 5139: 5134: 5124: 5123: 5119: 5118: 5106: 5083: 5082: 5080: 5079: 5066: 5053: 5040: 5027: 5014: 5001: 4987: 4985: 4981: 4980: 4978: 4977: 4972: 4967: 4962: 4957: 4951: 4949: 4945: 4944: 4942: 4941: 4936: 4931: 4926: 4920: 4918: 4917:Classification 4914: 4913: 4911: 4910: 4905: 4900: 4895: 4889: 4887: 4883: 4882: 4880: 4879: 4874: 4869: 4864: 4859: 4854: 4849: 4844: 4838: 4836: 4832: 4831: 4826: 4824: 4823: 4816: 4809: 4801: 4792: 4791: 4789: 4788: 4787: 4786: 4781: 4771: 4766: 4757: 4743: 4738: 4733: 4732: 4731: 4726: 4716: 4711: 4706: 4700: 4698: 4694: 4693: 4691: 4690: 4685: 4679: 4677: 4671: 4670: 4668: 4667: 4662: 4657: 4652: 4646: 4644: 4637: 4636: 4634: 4633: 4628: 4627: 4626: 4621: 4611: 4606: 4601: 4595: 4593: 4587: 4586: 4584: 4583: 4582: 4581: 4576: 4566: 4554: 4553: 4552: 4551: 4546: 4541: 4536: 4531: 4526: 4521: 4516: 4511: 4506: 4501: 4496: 4491: 4486: 4481: 4476: 4466: 4461: 4456: 4451: 4446: 4441: 4432: 4430: 4424: 4423: 4421: 4420: 4419: 4418: 4413: 4403: 4398: 4393: 4387: 4385: 4379: 4378: 4363:Endopeptidases 4361: 4359: 4358: 4351: 4344: 4336: 4327: 4326: 4323: 4322: 4320: 4319: 4314: 4309: 4304: 4302: 4296: 4295: 4293: 4292: 4287: 4282: 4276: 4274: 4268: 4267: 4265: 4264: 4259: 4254: 4248: 4246: 4240: 4239: 4237: 4236: 4231: 4225: 4223: 4214: 4208: 4207: 4205: 4204: 4199: 4197:-Macroglobulin 4194: 4190: 4185: 4181: 4176: 4171: 4166: 4161: 4156: 4150: 4148: 4142: 4141: 4139: 4138: 4133: 4128: 4123: 4118: 4113: 4107: 4105: 4099: 4098: 4095: 4094: 4092: 4091: 4086: 4081: 4067: 4062: 4057: 4052: 4046: 4044: 4042:Common pathway 4038: 4037: 4035: 4034: 4029: 4023: 4021: 4013: 4012: 4010: 4009: 4004: 3999: 3994: 3989: 3984: 3979: 3974: 3968: 3966: 3958: 3957: 3955: 3954: 3949: 3943: 3934: 3928: 3923: 3908: 3902: 3900: 3889: 3883: 3882: 3877: 3875: 3874: 3867: 3860: 3852: 3843: 3842: 3840: 3839: 3833: 3826: 3824: 3818: 3811: 3809: 3803: 3796: 3794: 3788: 3781: 3779: 3773: 3766: 3764: 3758: 3751: 3749: 3743: 3736: 3734: 3728: 3721: 3719: 3713: 3706: 3703: 3700: 3699: 3696: 3694: 3693: 3686: 3679: 3671: 3665: 3664: 3647: 3642: 3619: 3618:External links 3616: 3614: 3613: 3576: 3547: 3487: 3458: 3415: 3385: 3383: 3380: 3377: 3376: 3351: 3310: 3279:(17): 1671–5. 3259: 3229: 3199: 3169: 3139: 3109: 3079: 3049: 3019: 2995:"Refixia EPAR" 2986: 2953: 2920: 2896:"Benefix EPAR" 2887: 2843: 2794: 2768: 2739: 2711: 2691:"Rixubis EPAR" 2679: 2664: 2625:(21): 6461–4. 2605: 2548: 2507: 2472: 2413: 2392:(8): 5734–41. 2372: 2351:(5): 3622–31. 2328: 2287: 2260: 2231:(6): 1528–38. 2208: 2177: 2125: 2107: 2089: 2068: 2046: 2045: 2043: 2040: 2011: 2010: 2007: 2004: 2001: 1998: 1995: 1992: 1989: 1986: 1983: 1980: 1977: 1960: 1959: 1957: 1956: 1942: 1940: 1934: 1933: 1929: 1928: 1926: 1925: 1921: 1919: 1913: 1912: 1909: 1903: 1902: 1883: 1882: 1880: 1879: 1870: 1856: 1854: 1848: 1847: 1843: 1842: 1840: 1839: 1835: 1833: 1827: 1826: 1824: 1823: 1805: 1803: 1797: 1796: 1793: 1787: 1786: 1767: 1766: 1764: 1763: 1752: 1738: 1736: 1730: 1729: 1725: 1724: 1722: 1721: 1717: 1715: 1709: 1708: 1706: 1705: 1687: 1685: 1679: 1678: 1675: 1669: 1668: 1649: 1648: 1646: 1645: 1635: 1633: 1627: 1626: 1622: 1621: 1619: 1618: 1614: 1612: 1606: 1605: 1600: 1598:administration 1592: 1591: 1588: 1582: 1581: 1562: 1561: 1559: 1558: 1544: 1542: 1536: 1535: 1531: 1530: 1528: 1527: 1523: 1521: 1515: 1514: 1512: 1511: 1493: 1491: 1485: 1484: 1481: 1475: 1474: 1448: 1445: 1394: 1391: 1329: 1326: 1280:signal peptide 1259: 1256: 1209: 1208: 1205: 1204: 1199: 1189: 1188: 1182: 1181: 1178: 1177: 1175: 1173: 1166: 1165: 1158: 1151: 1147: 1146: 1143: 1142: 1128: 1127: 1121: 1118: 1117: 1103: 1102: 1096: 1092: 1091: 1088: 1087: 1073: 1072: 1066: 1063: 1062: 1048: 1047: 1041: 1037: 1036: 1033: 1032: 1024: 1023: 1017: 1014: 1013: 1005: 1004: 998: 992: 991: 988: 987: 979: 978: 972: 969: 968: 960: 959: 953: 947: 946: 943: 942: 934: 933: 927: 924: 923: 915: 914: 908: 902: 901: 896: 891: 887: 886: 876: 875: 872: 871: 860: 859: 857: 856: 851: 846: 841: 836: 831: 826: 821: 816: 810: 808: 804: 803: 801: 800: 795: 790: 785: 780: 775: 770: 764: 762: 758: 757: 755: 754: 749: 744: 739: 734: 729: 724: 719: 713: 711: 707: 706: 696: 695: 692: 691: 688: 687: 679: 678: 667: 661: 660: 657: 656: 648: 647: 644: 643: 641: 640: 636: 632: 628: 624: 620: 616: 612: 608: 604: 600: 597: 596: 585: 582: 581: 579: 578: 574: 570: 566: 562: 558: 554: 550: 546: 542: 538: 535: 534: 522: 521: 513: 502: 496: 495: 492:RNA expression 484: 483: 480: 479: 471: 467: 466: 458: 455: 450: 444: 443: 434: 427: 421: 417: 416: 411: 405: 404: 396: 395: 383: 382: 379: 378: 370: 366: 365: 357: 354: 349: 343: 342: 333: 326: 320: 316: 315: 308: 302: 301: 293: 292: 280: 279: 276:F9 - orthologs 236: 232: 231: 223: 215: 214: 210: 209: 206: 205: 202: 201: 71: 70: 62: 61: 50: 44: 43: 35: 34: 26: 25: 17: 13: 10: 9: 6: 4: 3: 2: 5184: 5173: 5170: 5168: 5165: 5163: 5160: 5158: 5155: 5153: 5150: 5148: 5145: 5143: 5140: 5138: 5135: 5133: 5130: 5129: 5127: 5117: 5107: 5105: 5100: 5095: 5091: 5077: 5073: 5072: 5067: 5064: 5060: 5059: 5054: 5051: 5047: 5046: 5041: 5038: 5034: 5033: 5028: 5025: 5021: 5020: 5015: 5012: 5008: 5007: 5002: 4999: 4995: 4994: 4989: 4988: 4986: 4982: 4976: 4973: 4971: 4968: 4966: 4963: 4961: 4958: 4956: 4953: 4952: 4950: 4946: 4940: 4937: 4935: 4934:Enzyme family 4932: 4930: 4927: 4925: 4922: 4921: 4919: 4915: 4909: 4906: 4904: 4901: 4899: 4898:Cooperativity 4896: 4894: 4891: 4890: 4888: 4884: 4878: 4875: 4873: 4870: 4868: 4865: 4863: 4860: 4858: 4857:Oxyanion hole 4855: 4853: 4850: 4848: 4845: 4843: 4840: 4839: 4837: 4833: 4829: 4822: 4817: 4815: 4810: 4808: 4803: 4802: 4799: 4785: 4782: 4780: 4777: 4776: 4775: 4772: 4770: 4769:Streptokinase 4767: 4765: 4761: 4758: 4755: 4751: 4747: 4744: 4742: 4739: 4737: 4734: 4730: 4727: 4725: 4722: 4721: 4720: 4717: 4715: 4712: 4710: 4707: 4705: 4702: 4701: 4699: 4695: 4689: 4686: 4684: 4681: 4680: 4678: 4676: 4672: 4666: 4663: 4661: 4658: 4656: 4653: 4651: 4648: 4647: 4645: 4643: 4642:immune system 4638: 4632: 4631:C3-convertase 4629: 4625: 4622: 4620: 4617: 4616: 4615: 4612: 4610: 4607: 4605: 4602: 4600: 4597: 4596: 4594: 4592: 4588: 4580: 4577: 4575: 4572: 4571: 4570: 4567: 4565: 4562: 4560: 4556: 4555: 4550: 4547: 4545: 4542: 4540: 4537: 4535: 4532: 4530: 4527: 4525: 4522: 4520: 4517: 4515: 4512: 4510: 4507: 4505: 4502: 4500: 4497: 4495: 4492: 4490: 4487: 4485: 4482: 4480: 4477: 4475: 4472: 4471: 4470: 4467: 4465: 4462: 4460: 4457: 4455: 4452: 4450: 4447: 4445: 4442: 4440: 4437: 4434: 4433: 4431: 4429: 4425: 4417: 4414: 4412: 4409: 4408: 4407: 4404: 4402: 4399: 4397: 4394: 4392: 4389: 4388: 4386: 4384: 4380: 4375: 4372: 4368: 4364: 4357: 4352: 4350: 4345: 4343: 4338: 4337: 4334: 4318: 4315: 4313: 4306: 4305: 4303: 4301: 4297: 4291: 4288: 4286: 4283: 4281: 4278: 4277: 4275: 4273: 4269: 4263: 4260: 4258: 4255: 4253: 4250: 4249: 4247: 4245: 4241: 4235: 4232: 4230: 4227: 4226: 4224: 4222: 4218: 4215: 4213: 4209: 4203: 4200: 4198: 4191: 4189: 4182: 4180: 4177: 4175: 4172: 4170: 4167: 4165: 4162: 4160: 4157: 4155: 4152: 4151: 4149: 4147: 4143: 4137: 4134: 4132: 4129: 4127: 4124: 4122: 4119: 4117: 4114: 4112: 4109: 4108: 4106: 4104: 4100: 4090: 4087: 4085: 4082: 4079: 4075: 4071: 4068: 4066: 4063: 4061: 4058: 4056: 4053: 4051: 4048: 4047: 4045: 4043: 4039: 4033: 4030: 4028: 4025: 4024: 4022: 4020: 4014: 4008: 4005: 4003: 4000: 3998: 3995: 3993: 3990: 3988: 3985: 3983: 3982:Prekallikrein 3980: 3978: 3975: 3973: 3970: 3969: 3967: 3965: 3959: 3953: 3950: 3947: 3944: 3942: 3938: 3935: 3932: 3929: 3927: 3924: 3922: 3918: 3915: 3913: 3909: 3907: 3904: 3903: 3901: 3899: 3893: 3890: 3888: 3884: 3880: 3873: 3868: 3866: 3861: 3859: 3854: 3853: 3850: 3836: 3830: 3825: 3821: 3815: 3810: 3806: 3800: 3795: 3791: 3785: 3780: 3776: 3770: 3765: 3761: 3755: 3750: 3746: 3740: 3735: 3731: 3725: 3720: 3716: 3710: 3705: 3701: 3692: 3687: 3685: 3680: 3678: 3673: 3672: 3669: 3663: 3659: 3656: 3652: 3648: 3646: 3643: 3640: 3636: 3635: 3630: 3626: 3622: 3621: 3617: 3610: 3606: 3602: 3598: 3594: 3590: 3586: 3582: 3577: 3573: 3569: 3565: 3561: 3557: 3553: 3548: 3534: 3530: 3526: 3522: 3518: 3513: 3508: 3505:(3): 507–13. 3504: 3500: 3493: 3488: 3484: 3480: 3476: 3472: 3468: 3464: 3459: 3455: 3451: 3447: 3443: 3438: 3433: 3429: 3425: 3424:FASEB Journal 3421: 3416: 3412: 3408: 3404: 3400: 3396: 3392: 3387: 3386: 3381: 3365: 3361: 3355: 3352: 3347: 3343: 3338: 3333: 3330:(3): 320–31. 3329: 3325: 3321: 3314: 3311: 3306: 3302: 3297: 3296:11577/2438365 3292: 3287: 3282: 3278: 3274: 3270: 3263: 3260: 3247: 3243: 3239: 3233: 3230: 3217: 3213: 3209: 3203: 3200: 3187: 3183: 3179: 3173: 3170: 3157: 3153: 3149: 3143: 3140: 3127: 3123: 3119: 3113: 3110: 3097: 3093: 3089: 3083: 3080: 3067: 3063: 3059: 3053: 3050: 3037: 3033: 3029: 3023: 3020: 3007: 3003: 3001: 2996: 2990: 2987: 2974: 2970: 2968: 2963: 2957: 2954: 2941: 2937: 2935: 2930: 2924: 2921: 2908: 2904: 2902: 2897: 2891: 2888: 2883: 2879: 2875: 2871: 2867: 2863: 2859: 2855: 2847: 2844: 2839: 2835: 2830: 2825: 2821: 2817: 2813: 2809: 2805: 2798: 2795: 2782: 2778: 2772: 2769: 2757:. 8 June 2007 2756: 2754: 2749: 2743: 2740: 2727: 2726: 2725:Health Canada 2721: 2715: 2712: 2699: 2697: 2692: 2686: 2684: 2680: 2675: 2668: 2665: 2660: 2656: 2651: 2646: 2641: 2636: 2632: 2628: 2624: 2620: 2616: 2609: 2606: 2601: 2597: 2592: 2587: 2583: 2579: 2575: 2571: 2567: 2563: 2559: 2552: 2549: 2544: 2540: 2535: 2530: 2526: 2522: 2518: 2511: 2508: 2503: 2499: 2495: 2491: 2487: 2483: 2476: 2473: 2468: 2464: 2459: 2454: 2449: 2444: 2440: 2436: 2432: 2428: 2424: 2417: 2414: 2409: 2405: 2400: 2395: 2391: 2387: 2383: 2376: 2373: 2368: 2364: 2359: 2354: 2350: 2346: 2342: 2335: 2333: 2329: 2324: 2320: 2315: 2310: 2306: 2302: 2298: 2291: 2288: 2283: 2279: 2276:(7): 685–93. 2275: 2271: 2264: 2261: 2256: 2252: 2247: 2242: 2238: 2234: 2230: 2226: 2222: 2215: 2213: 2209: 2203: 2198: 2194: 2190: 2184: 2182: 2178: 2173: 2169: 2164: 2159: 2155: 2151: 2147: 2143: 2139: 2132: 2130: 2126: 2121: 2117: 2111: 2108: 2103: 2099: 2093: 2090: 2086: 2082: 2077: 2075: 2073: 2069: 2065: 2061: 2056: 2054: 2052: 2048: 2041: 2039: 2037: 2032: 2029: 2027: 2022: 2020: 2016: 2008: 2005: 2002: 1999: 1996: 1993: 1990: 1987: 1984: 1981: 1978: 1975: 1974: 1973: 1971: 1967: 1954: 1944: 1943: 1941: 1939: 1935: 1930: 1923: 1922: 1920: 1918: 1914: 1910: 1908: 1904: 1901:Clinical data 1899: 1878: Rx-only 1871: 1868: 1858: 1857: 1855: 1853: 1849: 1844: 1837: 1836: 1834: 1832: 1828: 1821: 1816: 1807: 1806: 1804: 1802: 1798: 1794: 1792: 1788: 1785:Clinical data 1783: 1762: 1753: 1750: 1740: 1739: 1737: 1735: 1731: 1726: 1719: 1718: 1716: 1714: 1710: 1703: 1698: 1689: 1688: 1686: 1684: 1680: 1676: 1674: 1670: 1667:Clinical data 1665: 1644: Rx-only 1637: 1636: 1634: 1632: 1628: 1623: 1616: 1615: 1613: 1611: 1607: 1604: 1601: 1599: 1593: 1589: 1587: 1583: 1580:Clinical data 1578: 1575:nonacog gamma 1556: 1546: 1545: 1543: 1541: 1537: 1532: 1525: 1524: 1522: 1520: 1516: 1509: 1504: 1495: 1494: 1492: 1490: 1486: 1482: 1480: 1476: 1473:Clinical data 1471: 1457: 1454: 1446: 1444: 1442: 1438: 1434: 1430: 1426: 1424: 1420: 1416: 1412: 1404: 1399: 1392: 1390: 1387: 1385: 1379: 1377: 1373: 1372:tissue factor 1364: 1360: 1358: 1354: 1350: 1346: 1342: 1338: 1334: 1327: 1325: 1322: 1320: 1315: 1313: 1309: 1305: 1301: 1297: 1293: 1289: 1285: 1281: 1277: 1269: 1264: 1257: 1255: 1253: 1249: 1245: 1240: 1238: 1237:haemophilia B 1234: 1230: 1226: 1222: 1219: 1215: 1203: 1198: 1194: 1190: 1187: 1183: 1176: 1174: 1171: 1167: 1163: 1159: 1156: 1152: 1148: 1141: 1139: 1135: 1129: 1125: 1122: 1116: 1114: 1110: 1104: 1100: 1097: 1093: 1086: 1084: 1080: 1074: 1070: 1067: 1061: 1059: 1055: 1049: 1045: 1042: 1040:RefSeq (mRNA) 1038: 1031: 1030: 1025: 1021: 1018: 1012: 1011: 1006: 1002: 999: 997: 993: 986: 985: 980: 976: 973: 967: 966: 961: 957: 954: 952: 948: 941: 940: 935: 931: 928: 922: 921: 916: 912: 909: 907: 903: 900: 897: 895: 892: 888: 885: 881: 877: 870: 866: 861: 855: 852: 850: 847: 845: 842: 840: 837: 835: 832: 830: 827: 825: 822: 820: 817: 815: 812: 811: 809: 806: 805: 799: 796: 794: 791: 789: 786: 784: 781: 779: 776: 774: 771: 769: 766: 765: 763: 760: 759: 753: 750: 748: 745: 743: 740: 738: 735: 733: 730: 728: 725: 723: 720: 718: 715: 714: 712: 709: 708: 705: 704:Gene ontology 701: 697: 685: 680: 676: 671: 668: 666: 662: 654: 649: 638: 634: 630: 627:dentate gyrus 626: 622: 618: 614: 610: 606: 602: 601: 598: 594: 589: 586: 576: 572: 568: 564: 560: 557:skin of thigh 556: 552: 548: 544: 540: 539: 536: 532: 527: 524: 523: 520: 518: 514: 512: 511: 507: 506: 503: 501: 497: 493: 489: 485: 477: 472: 468: 464: 459: 449: 445: 438: 431: 425: 418: 410: 406: 402: 397: 393: 388: 384: 376: 371: 367: 363: 358: 348: 344: 337: 330: 324: 317: 313: 307: 303: 299: 294: 290: 285: 281: 277: 273: 269: 265: 261: 257: 253: 249: 245: 241: 233: 228: 221: 216: 211: 200: 198: 194: 190: 186: 182: 178: 174: 170: 166: 162: 158: 154: 150: 146: 142: 138: 134: 130: 126: 122: 118: 114: 110: 106: 102: 98: 94: 90: 86: 82: 78: 72: 67: 64: 63: 59: 56: 49: 45: 40: 36: 32: 27: 22: 16: 5071:Translocases 5068: 5055: 5042: 5029: 5016: 5006:Transferases 5003: 4990: 4847:Binding site 4559:fibrinolysis 4557: 4448: 4435: 4401:Chymotrypsin 4317:D-Dimer (DD) 4300:Fibrinolysis 4188:-Antiplasmin 4001: 3910: 3834: 3819: 3804: 3789: 3774: 3759: 3744: 3729: 3714: 3632: 3587:(3): 262–9. 3584: 3580: 3555: 3551: 3540:. Retrieved 3502: 3498: 3466: 3462: 3427: 3423: 3394: 3390: 3368:. Retrieved 3354: 3327: 3323: 3313: 3276: 3272: 3262: 3250:. Retrieved 3241: 3232: 3220:. Retrieved 3211: 3202: 3190:. Retrieved 3181: 3172: 3160:. Retrieved 3151: 3142: 3130:. Retrieved 3121: 3112: 3100:. Retrieved 3091: 3082: 3070:. Retrieved 3061: 3052: 3040:. Retrieved 3031: 3022: 3010:. Retrieved 2998: 2989: 2977:. Retrieved 2965: 2956: 2944:. Retrieved 2932: 2923: 2911:. Retrieved 2899: 2890: 2857: 2853: 2846: 2811: 2807: 2797: 2787:15 September 2785:. Retrieved 2780: 2771: 2759:. Retrieved 2751: 2742: 2730:. Retrieved 2723: 2714: 2702:. Retrieved 2694: 2673: 2667: 2622: 2618: 2608: 2568:(1): 18577. 2565: 2561: 2551: 2524: 2520: 2510: 2485: 2482:Biochemistry 2481: 2475: 2430: 2426: 2416: 2389: 2385: 2375: 2348: 2344: 2304: 2300: 2290: 2273: 2269: 2263: 2228: 2224: 2202:10665/325771 2192: 2145: 2141: 2119: 2110: 2101: 2092: 2033: 2030: 2023: 2012: 1966:hemophilia B 1963: 1938:Legal status 1932:Legal status 1852:Legal status 1846:Legal status 1801:License data 1734:Legal status 1728:Legal status 1683:License data 1631:Legal status 1625:Legal status 1540:Legal status 1534:Legal status 1489:License data 1468:nonacog alfa 1456: 1427: 1415:X chromosome 1409:Because the 1408: 1403:X chromosome 1388: 1380: 1375: 1374:. Wilkinson 1369: 1331: 1323: 1319:antithrombin 1316: 1299: 1284:glycosylated 1273: 1241: 1231:involved in 1224: 1213: 1212: 1134:NP_001292726 1131: 1113:NP_001300842 1106: 1083:NM_001305797 1076: 1058:NM_001313913 1051: 1027: 1008: 982: 963: 937: 918: 898: 893: 515: 508: 373:139,563,459 360:139,530,739 235:External IDs 74: 15: 4842:Active site 4464:Factor XIIa 4444:Factor VIIa 4428:Coagulation 4154:Plasminogen 4089:Factor XIII 4007:Factor VIII 3697:PDB gallery 3397:: 799–829. 2860:(1): 63–5. 1970:Recombinant 1907:Trade names 1791:Trade names 1673:Trade names 1603:Intravenous 1586:Trade names 1479:Trade names 1433:Poll Dorset 1333:Factors VII 1292:factor VIIa 1248:haemophilia 1233:coagulation 849:proteolysis 778:Golgi lumen 611:gallbladder 474:59,076,119 461:59,044,824 213:Identifiers 5126:Categories 5045:Isomerases 5019:Hydrolases 4886:Regulation 4746:Subtilisin 4688:Batroxobin 4469:Kallikrein 4459:Factor XIa 4449:Factor IXa 4416:Pancreatic 4411:Neutrophil 4032:Factor VII 3987:Kallikrein 3977:Bradykinin 3542:2019-12-11 3370:2020-10-23 2087:, May 2017 2066:, May 2017 2042:References 1451:See also: 1437:Ian Wilmut 1423:Earl Davie 1353:EGF domain 1349:Gla domain 1335:, IX, and 1308:isoleucine 1300:factor IXa 1288:factor XIa 1258:Physiology 814:hemostasis 519:(ortholog) 256:HomoloGene 5147:EC 3.4.21 4924:EC number 4774:Cathepsin 4760:Sedolisin 4736:Prostasin 4454:Factor Xa 4308:Plasmin-α 4169:Urokinase 4002:Factor IX 3997:Factor XI 2521:Structure 1888:Factor IX 1772:Factor IX 1654:Factor IX 1596:Routes of 1567:Factor IX 1460:Factor IX 1443:in 1997. 1268:Protein C 1221:3.4.21.22 1214:Factor IX 1138:NP_032005 1109:NP_000124 1079:NM_007979 1054:NM_000133 884:Orthologs 264:GeneCards 5162:Zymogens 5116:Medicine 4948:Kinetics 4872:Cofactor 4835:Activity 4675:Venombin 4660:Tryptase 4655:Granzyme 4609:Factor I 4604:Factor D 4599:Factor B 4439:Thrombin 4436:factors: 4406:Elastase 4055:Factor V 4050:Factor X 3658:Archived 3609:39904506 3601:17414217 3572:14567539 3533:Archived 3529:44650866 3521:11736930 3454:15211597 3364:Archived 3346:21712351 3305:19846852 3252:23 March 3246:Archived 3242:DailyMed 3222:23 March 3216:Archived 3212:DailyMed 3192:23 March 3186:Archived 3182:DailyMed 3162:23 March 3156:Archived 3152:DailyMed 3132:23 March 3126:Archived 3122:DailyMed 3102:23 March 3096:Archived 3092:DailyMed 3072:23 March 3066:Archived 3062:DailyMed 3042:23 March 3036:Archived 3032:DailyMed 3006:Archived 2973:Archived 2940:Archived 2907:Archived 2882:25251813 2838:25851415 2600:31819097 2543:20004170 2408:11714704 2367:11723140 2191:(2019). 2172:12997790 2083:– 2062:– 1917:ATC code 1831:ATC code 1815:DailyMed 1795:Alprolix 1713:ATC code 1697:DailyMed 1677:Idelvion 1610:ATC code 1519:ATC code 1503:DailyMed 1393:Genetics 1312:factor X 1310:bond in 1304:arginine 1270:pathway. 1186:Wikidata 863:Sources: 5104:Biology 5090:Portals 5058:Ligases 4828:Enzymes 4714:Pronase 4704:Acrosin 4650:Chymase 4564:Plasmin 4396:Trypsin 4159:Plasmin 3655:S01.214 3639:PDBe-KB 3629:UniProt 3483:9834200 3446:1634040 3012:17 June 2979:17 June 2946:17 June 2913:17 June 2874:1615485 2829:4496316 2732:7 April 2659:6959130 2627:Bibcode 2591:6901466 2570:Bibcode 2502:7547952 2467:7568220 2435:Bibcode 2323:7662969 2282:9331959 2163:2022306 2085:Ensembl 2064:Ensembl 2017:. This 1911:Refixia 1817::  1759:: 1699::  1590:Rixubis 1505::  1483:Benefix 1439:at the 1357:trypsin 1276:zymogen 996:UniProt 951:Ensembl 890:Species 869:QuickGO 639:jejunum 494:pattern 220:Aliases 5167:Sanofi 5032:Lyases 4741:Reelin 4683:Ancrod 4640:Other 4374:3.4.21 3946:GPIIIa 3651:MEROPS 3634:P00740 3607:  3599:  3570:  3527:  3519:  3481:  3452:  3444:  3411:237463 3409:  3344:  3303:  2880:  2872:  2836:  2826:  2761:7 June 2704:1 June 2657:  2650:347146 2647:  2598:  2588:  2541:  2500:  2465:  2455:  2406:  2365:  2321:  2280:  2255:659613 2253:  2246:372679 2243:  2170:  2160:  1951: 1865: 1813:  1761:â„ž-only 1747: 1695:  1553: 1501:  1347:: the 1172:search 1170:PubMed 1029:P16294 1010:P00740 906:Entrez 665:BioGPS 577:muscle 561:kidney 352:Xq27.1 244:300746 4984:Types 4750:Furin 4697:Other 4624:MASP2 4619:MASP1 4549:KLK15 4544:KLK14 4539:KLK13 4534:KLK12 4529:KLK11 4524:KLK10 3941:GPIIb 3926:GP1BB 3921:GP1BA 3605:S2CID 3536:(PDF) 3525:S2CID 3495:(PDF) 3463:Blood 3450:S2CID 3002:(EMA) 2969:(EMA) 2936:(EMA) 2903:(EMA) 2878:S2CID 2755:(EMA) 2698:(EMA) 2458:40889 2301:Blood 1429:Polly 1376:et al 939:14071 899:Mouse 894:Human 865:Amigo 565:blood 549:gonad 517:Mouse 510:Human 457:Start 392:Mouse 356:Start 289:Human 252:88384 5076:list 5069:EC7 5063:list 5056:EC6 5050:list 5043:EC5 5037:list 5030:EC4 5024:list 5017:EC3 5011:list 5004:EC2 4998:list 4991:EC1 4764:TPP1 4614:MASP 4519:KLK9 4514:KLK8 4509:KLK7 4504:KLK6 4499:KLK5 4494:KLK4 4489:KLK3 4484:KLK2 4479:KLK1 3835:1rfn 3820:1nl0 3805:1mgx 3790:1j35 3775:1j34 3760:1ixa 3745:1edm 3730:1cfi 3715:1cfh 3649:The 3627:for 3597:PMID 3568:PMID 3517:PMID 3479:PMID 3442:PMID 3407:PMID 3342:PMID 3301:PMID 3254:2024 3224:2024 3194:2024 3164:2024 3134:2024 3104:2024 3074:2024 3044:2024 3014:2020 2981:2020 2948:2020 2915:2020 2870:PMID 2834:PMID 2789:2024 2763:2024 2734:2024 2706:2024 2655:PMID 2596:PMID 2539:PMID 2498:PMID 2463:PMID 2404:PMID 2363:PMID 2319:PMID 2278:PMID 2251:PMID 2168:PMID 1924:None 1838:None 1720:None 1617:None 1526:None 1411:gene 920:2158 500:Bgee 448:Band 409:Chr. 347:Band 306:Chr. 240:OMIM 197:5JBA 193:5JB8 189:5JBB 185:5JB9 181:5JBC 177:5EGM 173:4WNH 169:4WN2 165:4WMK 161:4WMI 157:4WMA 153:4WM0 149:4ZAE 145:4Z0K 141:4YZU 137:3LC5 133:3LC3 129:3KCG 125:2WPM 121:2WPL 117:2WPK 113:2WPJ 109:2WPI 105:2WPH 101:1RFN 97:1NL0 93:1MGX 89:1IXA 85:1EDM 81:1CFI 77:1CFH 58:RCSB 55:PDBe 4754:S1P 4474:PSA 4078:FGG 4074:FGA 3625:PDB 3589:doi 3560:doi 3507:doi 3503:115 3471:doi 3432:doi 3399:doi 3332:doi 3291:hdl 3281:doi 3277:361 2862:doi 2824:PMC 2816:doi 2645:PMC 2635:doi 2586:PMC 2578:doi 2529:doi 2490:doi 2453:PMC 2443:doi 2394:doi 2390:277 2353:doi 2349:277 2309:doi 2241:PMC 2233:doi 2197:hdl 2158:PMC 2150:doi 1892:INN 1776:INN 1658:INN 1571:INN 1464:INN 1384:NMR 470:End 369:End 272:OMA 260:106 248:MGI 48:PDB 5128:: 4371:EC 4365:: 4076:, 3631:: 3603:. 3595:. 3585:14 3583:. 3566:. 3556:14 3554:. 3531:. 3523:. 3515:. 3501:. 3497:. 3477:. 3467:92 3465:. 3448:. 3440:. 3426:. 3422:. 3405:. 3395:44 3393:. 3362:. 3340:. 3328:13 3326:. 3322:. 3299:. 3289:. 3275:. 3271:. 3240:. 3210:. 3180:. 3150:. 3120:. 3090:. 3060:. 3030:. 2997:. 2964:. 2931:. 2898:. 2876:. 2868:. 2858:67 2856:. 2832:. 2822:. 2812:13 2810:. 2806:. 2779:. 2750:. 2722:. 2693:. 2682:^ 2653:. 2643:. 2633:. 2623:79 2621:. 2617:. 2594:. 2584:. 2576:. 2564:. 2560:. 2537:. 2525:17 2523:. 2519:. 2496:. 2486:34 2484:. 2461:. 2451:. 2441:. 2431:92 2429:. 2425:. 2402:. 2388:. 2384:. 2361:. 2347:. 2343:. 2331:^ 2317:. 2305:86 2303:. 2299:. 2274:62 2272:. 2249:. 2239:. 2229:61 2227:. 2223:. 2211:^ 2180:^ 2166:. 2156:. 2144:. 2140:. 2128:^ 2118:. 2100:. 2071:^ 2050:^ 2038:. 1953:S4 1947:AU 1874:EU 1867:S4 1861:AU 1810:US 1756:CA 1749:S4 1743:AU 1692:US 1640:EU 1555:S4 1549:AU 1498:US 1425:. 1386:. 1321:. 1282:, 1254:. 1239:. 1218:EC 867:/ 476:bp 463:bp 375:bp 362:bp 270:; 268:F9 266:: 262:; 258:: 254:; 250:: 246:; 242:: 227:F9 195:, 191:, 187:, 183:, 179:, 175:, 171:, 167:, 163:, 159:, 155:, 151:, 147:, 143:, 139:, 135:, 131:, 127:, 123:, 119:, 115:, 111:, 107:, 103:, 99:, 95:, 91:, 87:, 83:, 79:, 24:F9 5092:: 5078:) 5074:( 5065:) 5061:( 5052:) 5048:( 5039:) 5035:( 5026:) 5022:( 5013:) 5009:( 5000:) 4996:( 4820:e 4813:t 4806:v 4784:G 4779:A 4762:/ 4756:4 4752:/ 4748:/ 4729:2 4724:1 4561:: 4376:) 4369:( 4355:e 4348:t 4341:v 4310:2 4195:2 4193:α 4186:2 4184:α 4080:) 4072:( 3948:) 3939:( 3933:) 3919:( 3914:: 3871:e 3864:t 3857:v 3690:e 3683:t 3676:v 3641:. 3611:. 3591:: 3574:. 3562:: 3545:. 3509:: 3485:. 3473:: 3456:. 3434:: 3428:6 3413:. 3401:: 3373:. 3348:. 3334:: 3307:. 3293:: 3283:: 3256:. 3226:. 3196:. 3166:. 3136:. 3106:. 3076:. 3046:. 3016:. 2983:. 2950:. 2917:. 2884:. 2864:: 2840:. 2818:: 2791:. 2765:. 2736:. 2708:. 2661:. 2637:: 2629:: 2602:. 2580:: 2572:: 2566:9 2545:. 2531:: 2504:. 2492:: 2469:. 2445:: 2437:: 2410:. 2396:: 2369:. 2355:: 2325:. 2311:: 2284:. 2257:. 2235:: 2199:: 2174:. 2152:: 2146:2 2122:. 2104:. 1949:: 1894:: 1876:: 1863:: 1778:: 1745:: 1660:: 1642:: 1573:: 1551:: 1466:: 1337:X 1306:- 1216:( 394:) 291:) 274::

Index


PDB
PDBe
RCSB
1CFH
1CFI
1EDM
1IXA
1MGX
1NL0
1RFN
2WPH
2WPI
2WPJ
2WPK
2WPL
2WPM
3KCG
3LC3
3LC5
4YZU
4Z0K
4ZAE
4WM0
4WMA
4WMI
4WMK
4WN2
4WNH
5EGM

Text is available under the Creative Commons Attribution-ShareAlike License. Additional terms may apply.

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