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Faktor XI

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Hämostaseologie

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Zusammenfassung

Faktor XI (FXI) spielt eine zentrale Rolle im Rahmen der Hämostase. Seine physiologische Funktion besteht im Wesentlichen darin, das durch Kontakt mit Gewebeaktivator ausgelöste Gerinnungsgeschehen zu beschleunigen und zu verstärken; zusätzlich wirkt FXI antifibrinolytisch. FXI propagiert somit die plasmatische Gerinnung und führt zu erhöhter Stabilität bereits gebildeter Fibringerinnsel.

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Literatur

  • Asakai R, Davie EW, Chung DW (1987) Organization of the gene for human factor XI. Biochemistry 26(23): 7221–7228

    Article  CAS  PubMed  Google Scholar 

  • Baglia FA, Walsh PN (2000) Thrombin-mediated feedback activation of factor XI on the activated platelet surface is preferred over contact activation by factor XIIa or factor XIa. J Biol Chem 275(27): 20514–20519

    Article  CAS  PubMed  Google Scholar 

  • Baglia FA, Jameson BA, Walsh PN (1991) Identification and chemical synthesis of a substrate binding site for factor IX on coagulation factor XIa. J Biol Chem 266(35): 24190–24197

    CAS  PubMed  Google Scholar 

  • Baglia FA, Jameson BA, Walsh PN (1992) Fine mapping of the high weight kininogen binding site on blood coagulation factor XI through the use of rationally designed synthetic analogs. J Biol Chem 267(6): 4247–4252

    CAS  PubMed  Google Scholar 

  • Baglia FA, Jameson BA, Walsh PN (1993) Identification and characterization of a binding site for factor XIIa in the Apple 4 domain of coagulation factor XI. J Biol Chem 268(6): 3838–3844

    CAS  PubMed  Google Scholar 

  • Baglia FA, Jameson BA, Walsh PN (1995) Identification and characterization of a binding site for platelets in the Apple 3 domain of coagulation factor XI. J Biol Chem 270(12): 6734–6740

    Article  CAS  PubMed  Google Scholar 

  • Baglia FA, Badellino KO, Ho DH et al. (1996) A binding site thrombin in the apple 1 domain of factor XI. J Biol Chem 271(7): 3652–3658

    Article  CAS  PubMed  Google Scholar 

  • Baglia FA, Badellino KO, Ho DH et al. (2000) A binding site for the kringle II domain of prothrombin in the apple 1 domain of factor XI. J Biol Chem 275(41): 31954–31962

    Article  CAS  PubMed  Google Scholar 

  • Baglia FA, Badellino KO, Li CQ et al. (2002) Factor XI binding to the platelet glycoprotein Ib-IX-V complex promotes factor XI activation by thrombin. J Biol Chem 277(3): 1662–1668

    Article  CAS  PubMed  Google Scholar 

  • Baglia FA, Gailani D, López JA et al. (2004a) Identification of a binding site for glycoprotein Ibα in the apple 3 domain of factor XI. J Biol Chem 279(44): 45470–45476

    Article  CAS  PubMed  Google Scholar 

  • Baglia FA, Shrimpton CN, Emsley J et al. (2004b) Factor XI interacts with the leucine-rich repeats of glycoprotein Ibα on the activated platelet. J Biol Chem 279(47): 49323–49329

    Article  CAS  PubMed  Google Scholar 

  • Bajzar L, Morser J, Nesheim M (1996a) TAFI, or plasma procarboxypeptidase B, couples the coagulation and fibrinolytic cascades through the thrombin-thrombomodulin complex. J Biol Chem 271(28): 16603–166608

    Article  CAS  PubMed  Google Scholar 

  • Bajzar L, Nesheim ME, Tracy PB (1996b) The profibrinolytic effect of activated protein C in clots formed from plasma is TAFI-dependent. Blood 88(6): 2093–2100

    CAS  PubMed  Google Scholar 

  • Bajzar L, Nesheim M, Morser J et al. (1998) Both cellular and soluble forms of thrombomodulin inhibit fibrinolysis by potentiating the activation of thrombin-activatable fibrinolysis inhibitor. J Biol Chem 273(5): 2792–2798

    Article  CAS  PubMed  Google Scholar 

  • Beeler DL, Marcum JA, Schiffman S et al. (1986) Interaction of factor XIa and antithrombin in the presence and absence of heparin. Blood 67(5): 1488–1492

    CAS  PubMed  Google Scholar 

  • Berliner JI, Rybicki AC, Kaplan RC et al. (2002) Elevated levels of factor XI are associated with cardiovascular disease in women. Throm. Res. 107(1–2): 55–60

    Article  CAS  Google Scholar 

  • Bern MM, Sahud M, Zhukov O et al. (2005) Treatment of factor XI inhibitor using recombinant activated factor VIIa. Haemophilia 11(1): 20–25

    Article  CAS  PubMed  Google Scholar 

  • Berrettini M, Schleef RR, Espana F et al. (1989) Interaction of type 1 plasminogen activator inhibitor with the enzymes of the contact activation system. J Biol Chem 264(20): 11738–11743

    CAS  PubMed  Google Scholar 

  • Billon S, LeNiger M, Escoffre-Barbe M et al. (2001) The use of recombinant factor VIIa (NovoSeven) in a patient with factor XI deficiency and a circulating anticoagulant. Blood Coagul Fibrinolysis 12(7): 551–553

    Article  CAS  PubMed  Google Scholar 

  • Bolton-Maggs PHB (1998) The management of factor XI deficiency. Haemophilia 4(4): 683–688

    Article  CAS  PubMed  Google Scholar 

  • Bolton-Maggs PHB (2000) Factor XI deficiency and its management. Haemophilia 6(Suppl.1): 100–109

    Article  PubMed  Google Scholar 

  • Bolton-Maggs PH, Peretz H, Butler R et al. (2004a) A common ancestral mutation (C128X) occurring in 11 non-Jewish families from the UK with factor XI deficiency. J Thromb Haemost 2(6): 918–24

    Article  CAS  PubMed  Google Scholar 

  • Bolton-Maggs PHB, Perry DJ, Chalmers EA et al. (2004b) The rare coagulation disorders — review with guidelines for management from the United Kingdom Haemophilia Centre Doctors’ Organisation. Haemophilia 10(5): 593–628

    Article  CAS  PubMed  Google Scholar 

  • Bombeli T, Mueller M, Haeberli A (1997) Anticoagulant properties of the vascular endothelium. Thromb Haemost 77(3): 408–423

    CAS  PubMed  Google Scholar 

  • Bouma BN, Meijers JCM (2000) Role of blood coagulation factor XI in downregulation of fibrinolysis. Curr Opin Hematol 7(5): 266–272

    Article  CAS  PubMed  Google Scholar 

  • Bouma BN, Mosnier LO, Meijers JC et al. (1999) Factor XI dependent and independent activation of thrombin activatable fibrinolysis inhibitor (TAFI) in plasma associated with clot formation. Thromb Haemost 82(6): 1703–1708

    CAS  PubMed  Google Scholar 

  • Braat EAM, Rijken DC (1999) The inactivation of single-chain urokinasetype plasminogen activator by thrombin may provide an additional explanation for the antifibrinolytic effect of factor XI. Thromb Haemost 81(4): 657

    CAS  PubMed  Google Scholar 

  • Brown SA (2005) What dose of recombinant activated factor VII should be used in patients with factor XI deficiency. Haemophilia 11(4): 430–431

    Article  CAS  PubMed  Google Scholar 

  • Brunnée T, La Porta C, Reddigari SR et al. (1993) Activation of factor XI in plasma is dependent on factor XII. Blood 81(3): 580–586

    PubMed  Google Scholar 

  • Canadian Hemophilia Society (2006): Factor XI deficiency. http://www.hemophilia.ca/en/bleeding-disorders/other-factor-deficiencies/factor-xi-deficiency—hemophilia-c/ — Cited 03/2009

    Google Scholar 

  • Castaman G, Giacomelli SH, Dragani A et al. (2008) Severe factor XI deficiency in the Abruzzo region of Italy is associated to different FXI gene mutations. Haematologica 93(6): 957–958

    Article  CAS  PubMed  Google Scholar 

  • Chanarin I (1989) Laboratory Haematology. An account of laboratory techniques. Churchill Livingstone, Oxford

    Google Scholar 

  • Cheng Q, Kantz J, Poffenberger G et al. (2008) Factor XI protein in human pancreas and kidney. Thromb Haemost 100(1): 158–160

    CAS  PubMed  Google Scholar 

  • Davie EW, Fujikawa K, Kisiel W (1991) The coagulation cascade: initiation, maintenance, and regulation. Biochemistry 30(43): 10363–10370

    Article  CAS  PubMed  Google Scholar 

  • Diamant J, Perez AR, Strachmann Bacal N et al. (2004) Factor XI deficiency. Einstein 2(2): 115–116

    Google Scholar 

  • Doggen CJ, Rosendaal FR, Meijers FC (2006) Levels of intrinsic coagulation factors ant the risk of myocardial infarction among men: Opposite ansd synergistic effects of factors XI and XII. Blood 198(13): 4045–4051

    Article  CAS  Google Scholar 

  • Dorfman R, Walsh PN (2001) Noncovalent interactions of the apple 4 domain that mediate coagulation factor XI homodimerization. J Biol Chem 276(9): 6429–6438

    Article  CAS  PubMed  Google Scholar 

  • Dossenbach-Glaninger A, Hopmeier P (2005) Coagulation factor XI: a database of mutations and polymorphism associated with factor XI deficiency, Update 26-Feb 2009. http://www.wienkav.at/kav/kar/texte_anzeigen.asp?ID=7137 — Cited 3/2009

    Google Scholar 

  • Drummond Z, Retzinger G (2003) Factor XI deficiency: a review. Lab Lines 9(2): http://pathology.uc.edu/LABLINES/V9I2.pdf — Cited 03/2009

    Google Scholar 

  • Eichinger S, Schönauer V, Weltermann A et al. (2004) Thrombin-activatable fibrinolysis inhibitor and the risk for recurrent venous thromboembolism. Blood 103(10): 3773–3776

    Article  CAS  PubMed  Google Scholar 

  • Fujikawa K, Chung W, Hendrickson LE et al. (1986) Amino acid sequence of human factor XI, a blood coagulation factor with four tandem repeats that are highly homologous with plasma prekallikrein. Biochemistry 25(9): 2417–2424

    Article  CAS  PubMed  Google Scholar 

  • Gailani D, Broze GJ (1991) Factor XI activation in a revised model of blood coagulation. Science 253(5022): 909–912

    Article  CAS  PubMed  Google Scholar 

  • Gailani D, Broze GJ (1993a) Factor XII-independent activation of factor XI in plasma: effects of sulfatides on tissue factor-induced coagulation. Blood 82(3): 813–819

    CAS  PubMed  Google Scholar 

  • Gailani D, Broze GJ (1993b) Effects of glycosaminoglycans on factor XI activation by thrombin. Blood Coagul Fibrinolysis 4(1): 15–20

    CAS  PubMed  Google Scholar 

  • Gailani D, Ho D, Sun M-F et al. (2001) Model for a factor IX activation complex on blood platelets: dimeric conformation of factor XIa is essential. Blood 97(10): 3117–3122

    Article  CAS  PubMed  Google Scholar 

  • Gascoigne EW, Dash CH, Gillanders KR (2007) Clinical experience with a factor XI (FXI) concentrate. J Thromb Haemost 5(Suppl.2): Abstract P-S-208

    Google Scholar 

  • Goldstein DB, Reich DE, Bradman N et al. (1999) Age estimates of two common mutations causing factor XI deficiency: Recent genetic drift is not necessary for elevated disease incidence among Ashkenazi Jews. Am. J Hum Genet 64(4): 1071–1075

    Article  CAS  PubMed  Google Scholar 

  • Greengard JS, Heeb MJ, Ersdal E et al. (1986) Binding of coagulation factor XI to washed human platelets. Biochemistry 25(13): 3884–3890

    Article  CAS  PubMed  Google Scholar 

  • Han X, Fiehler R, Broze GJ Jr (2000) Characterization of the proteinZ-dependent protease inhibitor. Blood 96(9): 3049–3055

    CAS  PubMed  Google Scholar 

  • Hancock JF, Wieland K, Pugh RE et al. (1991) A molecular genetic study of factor XI deficiency. Blood 77(9): 1942–1948

    CAS  PubMed  Google Scholar 

  • Heck LW, Kaplan AP (1974) Substrates of Hageman factor. I. Isolation and characterization of human factor XI (PTA) and inhibition of the activated enzyme by alpha 1-antitrypsin. J Exp Med 140(6): 1615–1630

    Article  CAS  PubMed  Google Scholar 

  • Hedner U (1990) Factor VIIa in the treatment of haemophilia. Blood Coagul Fibrinolysis 1(3): 307–317

    Article  CAS  PubMed  Google Scholar 

  • Ho DH, Badellino K, Baglia FA et al. (1998) A binding site for heparin in the apple 3 domain of factor XI. J Biol Chem 273(26): 16382–16390

    Article  CAS  PubMed  Google Scholar 

  • Hopmeier P (2004) F XI deficiency. In: Fuchs J, Podda M (Ed) Encyclopedia of medical genomics and proteomics. Informa Health Care, London, New York, Melbourne, Stockholm. DOI 10.1081/E-EDGP-120020582

    Google Scholar 

  • Hsu TC, Shore SK, Seshsmma T et al. (1998) Molecular cloning of platelet factor XI, an alternative splicing product of the plasma factor XI gene. J Biol Chem 273(22): 13787–13793

    Article  CAS  PubMed  Google Scholar 

  • Keularts IMLW, Zivelin A, Seligsohn U et al. (2001) The role of factor XI in thrombin generation induced by low concentrations of tissue factor. Thromb Haemost 85(6): 1060–1065

    CAS  PubMed  Google Scholar 

  • Kolde H-J (2001) Haemostasis. Physiology, Pathology, Diagnostics. Pentapharm Ltd., Basel

    Google Scholar 

  • Kratz A, Ferraro M, Sluss PM et al. (2004) Laboratory reference values. N Engl J Med 351(15): 1548–1563

    Article  CAS  PubMed  Google Scholar 

  • Kravtsov DV, Wu W, Meijers JCM et al. (2004) Dominant factor XI deficiency caused by mutations in the factor XI catalytic domain. Blood 104(1): 128–134

    Article  CAS  PubMed  Google Scholar 

  • Kravtsov DV, Monahan PE, Gailani D (2005) A classification system for cross-reactive material-negative factor XI deficiency. Blood 105(12): 4671–4673

    Article  CAS  PubMed  Google Scholar 

  • Landes Bioscience 2000–2005. Eurekah Bioscience Collection. Coagulation. Factor XI, TAFI and DIC. http://www.ncbi.nlm.nih.gov/books/bv.fcgi?rid=eurekah.chapter.491 — Cited 03/2009

    Google Scholar 

  • Lavigne G, Mercier E, Queré I et al. (2003) Thrombnophilic families with inheritably associated high levels of coagulation factors VIII, IX and XI. J Thromb Haemost 1(10): 2134–2139

    Article  CAS  PubMed  Google Scholar 

  • Lawler P, White B, Pye S et al. (2002) Successful use of recombinant factor VIIa in a patient with inhibitor secondary to severe factor XI deficiency. Haemophilia 8(2): 145–148

    Article  CAS  PubMed  Google Scholar 

  • Libourel EJ, Bank I, Meinardi JR et al. (2002) Co-segregation of thrombophilic disorders in factor V Leiden carriers; the contributions of factor VIII, factor XI, thrombin activatable fibrinolysis inhibitor and lipoprotein(a) to the absolute risk of venous thromboembolism. Haematologica 87(10): 1068–1073

    CAS  PubMed  Google Scholar 

  • Mannhalter C, Hellstern P, Deutsch E (1987) Identification of a defective factor XI cross-reacting material in a factor XI-deficient patient. Blood 70(1): 31–37

    CAS  PubMed  Google Scholar 

  • Mannucci PM, Bauer KA, Santagostino E et al. (1994) Activation of the coagulation cascade after infusion of a factor XI concentrate in congenitally deficient patients. Blood 84(4): 1314–1319

    CAS  PubMed  Google Scholar 

  • Martincic D, Kravtsov V, Gailani D (1999) Factor XI messenger RNA in human platelets. Blood s94(10): 3397–3404

    Google Scholar 

  • McMullen BA, Fujikawa K, Davie EW (1991) Location of the disulfide bonds in human coagulation factor XI: The presence of tandem apple domains. Biochemistry 30(8): 2056–2060

    Article  CAS  PubMed  Google Scholar 

  • Meijers JC, Vlooswijk RA, Bouma BN (1988a) Inhibition of human blood coagulation factor XIa by C-1 inhibitor. Biochemistry 27(3): 959–963

    Article  CAS  PubMed  Google Scholar 

  • Meijers JC, Kanters DH, Vlooswijk RA et al. (1988b) Inactivation of human plasma kallikrein and factor XIa by protein C inhibitor. Biochemistry 27(12): 4231–4237

    Article  CAS  PubMed  Google Scholar 

  • Meijers JCM, Davie EW, Chung DW (1992a) Expression of human blood coagulation factor XI: Characterization of the defect in factor XI type III deficiency. Blood 79(6): 1435–1440

    CAS  PubMed  Google Scholar 

  • Meijers JC, Mulvihill ER, Davie EW et al. (1992b) Apple four in human blood coagulation factor XI mediates dimer formation. Biochemistry 31(19): 4680–4684

    Article  CAS  PubMed  Google Scholar 

  • Meijers JC, Tekelenburg WL, Bouma BN et al. (2000) High levels of coagulation factor XI as a risk factor for venous thrombosis. N Engl J Med 342(10): 696–701

    Article  CAS  PubMed  Google Scholar 

  • Merlo C, Wuillemin WA, Redondo M et al. (2002) Elevated levels of plasma prekallikrein, high moleculaer weight kininogen and factor XI in coronary heart disease. Atherosklerosis 161(2): 261–267

    Article  CAS  Google Scholar 

  • Minnema MC, ten Cate H, Hack CE (1999) The role of factor XI in coagulation: A matter of revision. Semin. Thromb. Hemost 25(4): 419–428

    Article  CAS  PubMed  Google Scholar 

  • Mitchell MJ, Dai L, Clarke JB et al. (2007) Characterisation of five factor XI mutations. Thromb Haemostas 97(6): 884–889

    CAS  Google Scholar 

  • Mutch NJ, Moore NR, Wang E et al. (2003) Thrombus lysis by uPA, scuPA and tPA is regulated by plasma TAFI. J Thromb Haemost 1(9): 2000–2007

    Article  CAS  PubMed  Google Scholar 

  • Naito K, Fujikawa K (1991) Activation of human blood coagulation factor XI independent of factor XII. J Biol Chem 266(12): 7353–7358

    CAS  PubMed  Google Scholar 

  • Navaneetham D, Jin L, Pandey P et al. (2005) Structural and mutational analyses of the molecular interactions between the catalytic domain of factor XIa and the Kunitz protease inhibitor domain of protease nexin 2. J Biol Chem 280(43): 36165–36175

    Article  CAS  PubMed  Google Scholar 

  • NCBI (2009) Entrez Gene. Update 11-Jan 2009. http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=retrieve&list_uids=2160. Cited 03/2009

    Google Scholar 

  • O’Connell NM (2003) Factor XI deficiency — from molecular genetics to clinical management. Blood Coagul Fibrinolysis 14(Suppl.1): S59–S64

    Article  PubMed  Google Scholar 

  • O’Connell NM (2004) Factor XI deficiency. Semin Hematol 41(1,Suppl.1): 76–81

    Article  PubMed  CAS  Google Scholar 

  • Papagrigoriou E, McEwan PA, Walsh PN et al. (2006) Crystal structure of the factor XI zymogen reveals a pathway for transactivation. Nat. Struct. Mol. Biol. 13(6): 557–558

    Article  CAS  PubMed  Google Scholar 

  • Peretz H, Mulai A, Usher S et al. (1997) The two common mutations causing factor XI deficiency in Jews stem from distinct founders: One of ancient Middle Eastern origin and another of more recent European origin. Blood 90(7): 2654–2659

    CAS  PubMed  Google Scholar 

  • Petersen LC, Sprecher CA, Foster DC et al. (1996) Inhibitory properties of a novel human Kunitz-type protease inhibitor homologous to tissue factor pathway inhibitor. Biochemistry 35(1): 266–272

    Article  CAS  PubMed  Google Scholar 

  • Podmore A, Smith M, Savidge G et al. (2004) Real-time quantitative analysis of factor XI mRNA variants in human platelets. J Thromb Haemost 2(10): 1713–1719

    Article  CAS  PubMed  Google Scholar 

  • Pugh RE, McVey JH, Tuddenham EGD et al. (1995) Six point mutations that cause factor XI deficiency. Blood 85(6): 1509–1516

    CAS  PubMed  Google Scholar 

  • Rakocz M, Mazar A, Varon D et al. (1993) Dental extractions in patients with bleeding disorders. The use of fibrin glue. Oral Surg Oral Med Oral Pathol 75(3): 280–282

    Article  CAS  PubMed  Google Scholar 

  • Rezaie AR, Sun MF, Gailani D (2006) Contributions of basic amino acids in the autolysis loop of factor XIa to serpin specificity. Biochemistry 45(31): 9427–9433

    Article  CAS  PubMed  Google Scholar 

  • Saito H, Goldsmith GH, Moroi M et al. (1979) Inhibitory spectrum of alpha 2-plasmin inhibitor. Proc Natl Acad Sci USA 76(4): 2013–2017

    Article  CAS  PubMed  Google Scholar 

  • Salomon O, Zivelin A, Levnat T et al. (2003) Prevalence, causes and characterization of factor XI inhibitors in patients with inherited factor XI deficiency. Blood 101(12): 4783–4788

    Article  CAS  PubMed  Google Scholar 

  • Salomon O, Steinberg DM, Tamarin I et al (2005) Plasma replacement therapy during labor is not mandatory for women with severe factor XI deficiency. Blood Coagul Fibrinolysis 16(1): 37–41

    Article  CAS  PubMed  Google Scholar 

  • Salomon O, Steinberg DM, Seligsohn U (2006) Variable bleeding manifestations characterize different types of surgery in patients with severe factor XI deficiency enabling parsimonious use of replacement therapy. Haemophilia 12(5): 490–493

    Article  CAS  PubMed  Google Scholar 

  • Salomon O, Steinberg DM, Tamarin I et al (2005) Plasma replacement therapy during labor is not mandatory for women with severe factor XI deficiency. Blood Coagul Fibrinolysis 16(1): 37–41

    Article  CAS  PubMed  Google Scholar 

  • Santagostino E, Mannucci PM, Bonomi AB (2000) Guidelines on replacement therapy for haemophilia and inherited coagulation disorders in Italy. Haemophilia 6(1): 1–10

    Article  CAS  PubMed  Google Scholar 

  • Santamaría A, Oliver A, Borrell M et al. (2007) Higher risk of ischaemic stroke associated with factor XI levels in dyslipidaemic patients. Int J Clin Pract 61(11): 1819–1823

    Article  PubMed  CAS  Google Scholar 

  • Saunders RE, Perkins SJ, O’Connell NM et al. (2006) F XI Deficiency Mutation Database. Version 2.1, updated Oct 2008. http://www.factorxi.org/ — Cited 03/2009

    Google Scholar 

  • Schulman S, Németh G (2006) An illustrative case and a review on the dosing of recombinant factor VIIa in congenital factor XI deficiency. Haemophilia 12(3): 223–227

    Article  CAS  PubMed  Google Scholar 

  • Scott CF, Colman RW (1989) Factors influencing the acceleration of human factor XIa inactivation by antithrombin III. Blood 73(7): 1873–1879

    CAS  PubMed  Google Scholar 

  • Seaman FS, Baglia FA, Gurr JA et al. (1994) Binding of high-molecularmass kininogen to the Apple 1 domain of factor XI is mediated in part by Val64 and Ile77. Biochem J 304 (Pt3): 715–721

    CAS  PubMed  Google Scholar 

  • Seligsohn U (1993) Factor XI deficiency. Thromb Haemost 70(1): 68–71

    CAS  PubMed  Google Scholar 

  • Seligsohn U (2007) Factor XI in haemostasis and thrombosis: Past, present and future. Thromb Haemost 98(1): 84–89

    CAS  PubMed  Google Scholar 

  • Siegel JE, Kouides PA (2002) Menorrhagia from a haematologist’s point of view. Part II: management. Haemophilia 8(3): 339–347

    Article  CAS  PubMed  Google Scholar 

  • Siegel JE (2006) Factor XI deficiency. eMedicine Journal, Updated: May 24, 2006. http://www.emedicine.com/med/topic3515.htm — Cited 03/2009

    Google Scholar 

  • Sinha D, Seaman FS, Koshy A et al. (1984) Blood coagulation factor XIa binds specifically to a site on activated human platelets distinct from that for factor XI. J Clin Invest 73(6): 1550–1556

    Article  CAS  PubMed  Google Scholar 

  • Soons H, Janssen-Claessen T, Tans G et al. (1987) Inhibition of factor XIa by antithrombin III. Biochemistry 26(15): 4624–4629

    Article  CAS  PubMed  Google Scholar 

  • Sun M-F, Zhao M, Gailani D (1999) Identification of amino acids in the factor XI apple 3 domain required for activation of factor IX. J Biol Chem 274(51): 36373–36378

    Article  CAS  PubMed  Google Scholar 

  • Sun M-F, Baglia FA, Ho D et al. (2001) Defective binding of factor XI-N248 to activated platelets. Blood 98(1): 125–129

    Article  CAS  PubMed  Google Scholar 

  • Tabatabai A, Fiehler R, Broze GJ Jr (2001) Protein Z circulates in plasma in a complex with protein Z-dependent protease inhibitor. Thromb Haemost 85(4): 655–660

    CAS  PubMed  Google Scholar 

  • Tanis B, Algra A, van der Graaf Y et al. (2006) Procoagulant factors and the risk of myocardial infarction in young women. Eur J Haematol 77(1): 67–73

    Article  CAS  PubMed  Google Scholar 

  • Tarumi T, Kravtsov DV, Zhao M et al. (2002) Cloning and Characterization of the human factor XI gene promoter. J Biol Chem 277(21): 18510–18516

    Article  CAS  PubMed  Google Scholar 

  • Tucker EI, Gailani D, Cheng Q et al. (2007) Reduced mortality of peritoneal sepsis in factor XI deficient mice. J Thromb Haemost 5(Suppl.2): Abstract P-W-011

    Google Scholar 

  • van der Graaf F, Greengard JS, Bouma BN et al. (1983) Isolation and functional characterization of the active light chain of activated human blood coagulation factor XI. J Biol Chem 258(16): 9669–9675

    PubMed  Google Scholar 

  • van Nostrand WE, Schmaier AH, Farrow JS et al. (1990) Protease nexin-II (amyloid beta-protein precursor): a platelet alpha-granule protein. Science 248(4956): 745–748

    Article  PubMed  Google Scholar 

  • Ventura C, Santos AIM, Tavares A et al. (2000) Molecular genetic analysis of factor XI deficiency: Identification of five novel gene alterations and the origin of type II mutation in Portuguese families. Thromb Haemost 84(5): 833–840

    CAS  PubMed  Google Scholar 

  • von dem Borne PA, Koppelman SJ, Bouma BN et al. (1994) Surface independent facor XI activation by thrombin in the presense of high molecular weight kininogen. Thromb Haemost 72(3): 397–402

    Google Scholar 

  • von dem Borne PA, Meijers JC, Bouma BN (1995) Feedback activation of factor XI by thrombin in plasma results in additional formation of thrombin that protects fibrin clots from fibrinolysis. Blood 86(8): 3035–3042

    Google Scholar 

  • von dem Borne PA, Meijers JC, Bouma BN (1996) Effect of heparin on the activation of factor XI by fibrin-bound thrombin. Thromb Haemost 76(3): 347–353

    Google Scholar 

  • von dem Borne PA, Cox LM, Bouma BN (2006) Factor XI enhances fibrin generarion and inhibits fibrinolysis in a coagulation model initiated by surface-coated tissue factor. Blood Coagul Fibrinolysis 17(4): 251–257

    Article  CAS  Google Scholar 

  • Walsh PN (2001) Roles of platelets and factor XI in the initiation of blood coagulation by thrombin. Thromb Haemost 86(1): 75–82

    CAS  PubMed  Google Scholar 

  • Wolberg A, Morris D, Stafford D (1997) Factor IX activation by factor XIa proceeds without release of a free intermediate. Biochemistry 36(14): 4074–4079

    Article  CAS  PubMed  Google Scholar 

  • Wuillemin WA, Mertens K, ten Cate H et al. (1996) Thrombin-mediated activation of endogenous factor XI in plasma in the presence of physiological glycosaminoglycans occurs only with high concentrations of thrombin. Br J Haematol 92(2): 466–472

    Article  CAS  PubMed  Google Scholar 

  • Yang DT, Flanders MM, Kim H et al. (2006) Elevated factor XI activity levels are associated with an increased odds ratio for cerebrovascular events. Am J Clin Pathol 126(3): 411–415

    Article  CAS  PubMed  Google Scholar 

  • Yun TH, Baglia FA, Myles T et al. (2003) Thrombin activation of factor XI on activated platelets requires the interaction of factor XI and platelet glycoprotein Ibα with thrombin anion binding exosites I and II, respectively. J Biol Chem 278(48): 48112–48119

    Article  CAS  PubMed  Google Scholar 

  • Zhang Y, Scandura JM, van Nostrand WE et al. (1997) The mechanism by which heparin promotes the inhibition of coagulation factor XIa by protease nexin-2. J Biol Chem 272(42): 26139–26144

    Article  CAS  PubMed  Google Scholar 

  • Zhang J, Tu Y, Lu L et al. (2008) Protein Z-dependent protease inhibitor deficiency produces a more severe murine phenotype than protein Z deficiency. Blood 111(10): 4973–4978

    Article  CAS  PubMed  Google Scholar 

  • Zhao M, Abdel-Razek T, Sun MF et al. (1998) Characterization of a heparin binding site on the heavy chain of factor XI. J Biol Chem 273(47): 31153–31159

    Article  CAS  PubMed  Google Scholar 

  • Zivelin A, Seligsohn U (2007) Mutations causing rare bleeding disorders. In: Supplement to chapter 116 of Williams Hematology 7th Edition 2006: Mutations in patients with factor XI deficiency. http://www.med.unc.edu/isth/mutations-databases/FactorXI_2007.htm. Updated February 15: 2007

    Google Scholar 

  • Zivelin A, Bauduer F, Ducout L et al. (2002) Factor XI deficiency in French Basques is caused predominantly by an ancestral Cys38Arg mutation in the factor XI gene. Blood 99(7): 2448–2454

    Article  CAS  PubMed  Google Scholar 

  • Zivelin A, Zucker M, Salomon O (2007) Factor XI phenotype-genotype correlations. J Thromb Haemost 5(Suppl.2): Abstract SYM-M-033

    Google Scholar 

  • Zucker M, Zivelin A, Landau M et al. (2007a) Three residues (L284, I290, Y329) in the apple 4 domain of factor XI (F XI) are essential for covalent dimerization of FXI subunits. J Thromb Haemost 5(Suppl.2): Abstract O-S-086

    Google Scholar 

  • Zucker M, Zivelin A, Teitel J et al. (2007b) Facor XI (FXI) is present in anti-D preparations and can induce an inhibitor to FXI in patients with severe FXI deficiency. J Thromb Haemost 5(Suppl.2): Abstract P-W-013

    Google Scholar 

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  1. Zentrallaboratorium und Blutbank, Krankenanstalt Rudolfstiftung, Juchgasse 25, A-1030, Wien

    Univ.-Doz. Dr. P. Hopmeier

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  1. Univ.-Doz. Dr. P. Hopmeier
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  1. Institut für Experimentelle Hämatologie und Transfusionsmedizin, Universitätsklinikum Bonn, Sigmund-Freud-Straße 25, 53105, Bonn

    Bernd Pötzsch

  2. Kerckhoff Klinik, BenekeStraße 2-8, 61231, Bad Nauheim

    Katharina Madlener

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Hopmeier, P. (2010). Faktor XI. In: Pötzsch, B., Madlener, K. (eds) Hämostaseologie. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-01544-1_20

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