Factor XIII is a key molecule at the intersection of coagulation and fibrinolysis as well as inflammation and infection control

International Journal of Hematology

Volumn 95, Issue 4, 2012, Pages 362-370

  Ichinose, Akitada ^a  

^a YAMAGATA UNIVERSITY   (Japan)

Author keywords

Anti fibrinolytic factor; Autoimmune hemorrhaphilia XIII 13; Fibrin stabilizing factor; Hereditary factor XIII B subunit deficiency; Innate immune factor; Plasma transglutaminase

Indexed keywords

BLOOD CLOTTING FACTOR 13; BLOOD CLOTTING FACTOR 13 CONCENTRATE; BLOOD CLOTTING FACTOR 13A; BLOOD CLOTTING FACTOR 13B; CATRIDECACOG; FIBRIN; MUTANT PROTEIN; PROTEIN ANTIBODY;

ARTICLE; BLEEDING; BLOOD CLOT RETRACTION; BLOOD CLOTTING; BLOOD CLOTTING FACTOR 13 DEFICIENCY; BLOOD CLOTTING FACTOR 13A DEFICIENCY; BLOOD CLOTTING FACTOR 13B DEFICIENCY; CELL SPREADING; FIBRINOLYSIS; HUMAN; IMMUNOSUPPRESSIVE TREATMENT; INFECTION CONTROL; INFLAMMATION; INNATE IMMUNITY; MACROPHAGE; NONHUMAN; PHAGOCYTOSIS; RARE DISEASE; THROMBOCYTE ADHESION;

ANIMALS; BLOOD COAGULATION; BLOOD COAGULATION DISORDERS; FACTOR XIII; HUMANS; IMMUNITY, INNATE;

EID: 84862854584     PISSN: 09255710     EISSN: 18653774     Source Type: Journal    
DOI: 10.1007/s12185-012-1064-3     Document Type: Article

References (54)

1. Muszbek L, Yee VC, Hevessy Z. Blood coagulation factor XIII: structure and function. Thromb Res. 1999;94:271-305.
2. Ichinose A. Physiopathology and regulation of factor XIII. Thromb Haemost. 2001;86:57-65.
3. Koseki S, Souri M, Koga S, Yamakawa M, Shichishima T, Maruyama Y, Yanai F, Ichinose A. Truncated mutant B subunit for factor XIII causes its deficiency due to impaired intracellular transportation. Blood. 2001;97:2667-72.
4. Ivaskevicius V, Biswas A, Loreth R, Schroeder V, Ohlenforst S, Rott H, Krause M, Kohler HP, Scharrer I, Oldenburg J. Mutations affecting disulphide bonds contribute to a fairly common prevalence of F13B gene defects: results of a genetic study in 14 families with factor XIII B deficiency. Haemophilia. 2010;16: 675-82.
5. Lauer P, Metzner HJ, Zettlmeissl G, Li M, Smith AG, Lathe R, Dickneite G. Targeted inactivation of the mouse locus encoding coagulation factor XIII-A: hemostatic abnormalities in mutant mice and characterization of the coagulation deficit. Thromb Haemost. 2002;88:967-74.
6. Souri M, Koseki-Kuno S, Takeda N, Degen JL, Ichinose A. Administration of factor XIII B subunit increased plasma factor XIII A subunit levels in factor XIII B subunit knock-out mice. Int J Hematol. 2008;87:60-8.
7. Kasahara K, Souri M, Kaneda M, Miki T, Yamamoto N, Ichinose A. Impaired clot retraction in factor XIII A subunit-deficient mice. Blood. 2010;115:1277-9.
8. Cohen I, Gerrard JM, White JG. Ultrastructure of clots during isometric contraction. J Cell Biol. 1982;93:775-87.
9. Woofter RT, Maurer MC. Role of calcium in the conformational dynamics of factor XIII activation examined by hydrogen-deuterium exchange coupled with MALDI-TOF MS. Arch Biochem Biophys. 2011;512:87-95.
10. Ono A, Westein E, Hsiao S, Nesbitt WS, Hamilton JR, Schoenwaelder SM, Jackson SP. Identification of a fibrin-independent platelet contractile mechanism regulating primary hemostasis and thrombus growth. Blood. 2008;112:90-9.
11. Rex S, Beaulieu LM, Perlman DH, Vitseva O, Blair PS, McComb ME, Costello CE, Freedman JE. Immune versus thrombotic stimulation of platelets differentially regulates signalling pathways, intracellular protein-protein interactions, and alpha-granule release. Thromb Haemost. 2009;102:97-110.
12. Ohmori T, Yatomi Y, Asazuma N, Satoh K, Ozaki Y. Involvement of proline-rich tyrosine kinase 2 in platelet activation: tyrosine phosphorylation mostly dependent on alphaIIbbeta3 integrin and protein kinase C, translocation to the cytoskeleton and association with Shc through Grb2. Biochem J. 2000;347:561-9.
13. Carr ME Jr. Development of platelet contractile force as a research and clinical measure of platelet function. Cell Biochem Biophys. 2003;38:55-78.
14. Léon C, Eckly A, Hechler B, Aleil B, Freund M, Ravanat C, Jourdain M, Nonne C, Weber J, Tiedt R, Gratacap MP, Severin S, Cazenave JP, Lanza F, Skoda R, Gachet C. Megakaryocyterestricted MYH9 inactivation dramatically affects hemostasis while preserving platelet aggregation and secretion. Blood. 2007; 110:3183-91.
15. Magwenzi SG, Ajjan RA, Standeven KF, Parapia LA, Naseem KM. Factor XIII supports platelet activation and enhances thrombus formation by matrix proteins under flow conditions. J Thromb Haemost. 2011;9:820-33.
16. Takizawa H, Nishimura S, Takayama N, Oda A, Nishikii H, Morita Y, Kakinuma S, Yamazaki S, Okamura S, Tamura N, Goto S, Sawaguchi A, Manabe I, Takatsu K, Nakauchi H, Takaki S, Eto K. Lnk regulates integrin alphaIIbbeta3 outside-in signaling in mouse platelets, leading to stabilization of thrombus development in vivo. J Clin Invest. 2010;120:179-90.
17. Randriamboavonjy V, Isaak J, Frömel T, Viollet B, Fisslthaler B, Preissner KT, Fleming I. AMPK a2 subunit is involved in platelet signaling, clot retraction, and thrombus stability. Blood. 2010; 116:2134-40.
18. Jayo A, Conde I, Lastres P, Jiménez-Yuste V, González- Mancho ́n C. New insights into the expression and role of platelet factor XIII-A. J Thromb Haemost. 2009;7:1184-91.
19. Nagy B Jr, Simon Z, Bagoly Z, Muszbek L, Kappelmayer J. Binding of plasma factor XIII to thrombin-receptor activated human platelets. Thromb Haemost. 2009;102:83-9.
20. Krarup A, Gulla KC, Gál P, Hajela K, Sim RB. The action of MBL-associated serine protease 1 (MASP1) on factor XIII and fibrinogen. Biochim Biophys Acta. 2008;1784:1294-300.
21. Gulla KC, Gupta K, Krarup A, Gal P, Schwaeble WJ, Sim RB, O'Connor CD, Hajela K. Activation of mannan-binding lectinassociated serine proteases leads to generation of a fibrin clot. Immunology. 2010;129:482-95.
22. Wang Z, Wilhelmsson C, Hyrsl P, Loof TG, Dobes P, Klupp M, Loseva O, Mörgelin M, Iklé J, Cripps RM, Herwald H, Theopold U. Pathogen entrapment by transglutaminase-a conserved early innate immune mechanism. PLoS Pathog. 2010;6:e1000763.
23. Loof TG, Mörgelin M, Johansson L, Oehmcke S, Olin AI, Dickneite G, Norrby-Teglund A, Theopold U, Herwald H. Coagulation, an ancestral serine protease cascade, exerts a novel function in early immune defense. Blood. 2011;118:2589-98.
24. Kawabata S, Muta T. Sadaaki Iwanaga: discovery of the lipopolysaccharide-and beta-1,3-D-glucan-mediated proteolytic cascade and unique proteins in invertebrate immunity. J Biochem. 2010;147:611-8.
25. Ding JL, Li P, Ho B. The Sushi peptides: structural characterization and mode of action against Gram-negative bacteria. Cell Mol Life Sci. 2008;65:1202-19.
26. Töröcsik D, Szeles L, Paragh G Jr, Rakosy Z, Bardos H, Nagy L, Balazs M, Inbal A, Adány R. Factor XIII-A is involved in the regulation of gene expression in alternatively activated human macrophages. Thromb Haemost. 2010;104:709-17.
27. Sárváry A, Szucs S, Balogh I, Becsky A, Bárdos H, Kávai M, Seligsohn U, Egbring R, Lopaciuk S, Muszbek L, Adány R. Possible role of factor XIII subunit A in Fcgamma and complement receptor-mediated phagocytosis. Cell Immunol. 2004;228:81-90.
28. Nahrendorf M, Sosnovik DE, Waterman P, Swirski FK, Pande AN, Aikawa E, Figueiredo JL, Pittet MJ, Weissleder R. Dual channel optical tomographic imaging of leukocyte recruitment and protease activity in the healing myocardial infarct. Circ Res. 2007;100:1218-25.
29. Brinkhous KM (1975) A short history of hemophilia, with some comments on the word Hemophilia. In: Brinkhous KM, Hemker HC (eds). Handbook of Hemophilia, Part 1, Amsterdam, New York: Excerpta Medica, American Elsevier Pub. Co.; 1975, pp 3-20
30. Boggio LN, Green D. Acquired hemophilia. Rev Clin Exp Hematol. 2001;5:389-404.
31. Franchini M, Lippi G. Acquired factor VIII inhibitors. Blood. 2008;112:250-5.
32. Collins PW, Hirsch S, Baglin TP, Dolan G, Hanley J, Makris M, for the UK Haemophilia Centre Doctors' Organisation. Acquired hemophilia A in the United Kingdom: a 2-year national surveillance study by the United Kingdom Haemophilia Centre Doctors' Organisation. Blood. 2007;109:1870-7.
33. Collins PW. Management of acquired haemophilia A. J Thromb Haemost. 2011;9(SUPPL. 1):226-35.
34. Ichinose A. Hemorrhagic acquired factor XIII (13) deficiency and acquired hemorrhaphilia 13 revisited. Semin Thromb Hemost. 2011;37:382-8.
35. Ichinose A, Japanese collaborative research group on Acquired haemorrhaphilia due to factor XIII deficiency. As many as 12 cases with haemorrhagic acquired factor XIII deficiency due to its inhibitors were recently found in Japan. Thromb Haemost. 2011; 105:925-7.
36. Lorand L. Acquired inhibitors of fibrin stabilization: a class of hemorrhagic disorders of diverse origins. In: Green D, editor. Anticoagulants, physiologic, pathologic and pharmacologic. Boca Raton: CRC Press; 1994. p. 169-91.
37. Egbring R, Kröniger A, Seitz R. Erworbene Inhibitoren gegen Faktor XIII. Hämostaseologie. 1996;16:174-9.
38. Ishida F, Okubo K, Ito T, Souri M, Ichinose A. Spontaneous regression of the inhibitor against the coagulation factor XIII A subunit in acquired factor XIII deficiency. Thromb Haemost. 2010;104:1284-5.
39. Ichinose A, Souri M. Reduced difference of a(2)-plasmin inhibitor levels between plasma and serum in patients with severe factor XIII deficiency, including autoimmune hemorrhaphilia due to anti-factor XIII antibodies. Int J Hematol. 2012;95:47-50.
40. Sakata Y, Aoki N. Significance of cross-linking of alpha2-plasmin inhibitor to fibrin in inhibition of fibrinolysis and in hemostasis. J Clin Invest. 1982;69:536-42.
41. Mutch NJ, Koikkalainen JS, Fraser SR, Duthie KM, Griffin M, Mitchell J, et al. Model thrombi formed under flow reveal the role of factor XIII-mediated cross-linking in resistance to fibrinolysis. J Thromb Haemost. 2010;8:2017-24.
42. Fraser SR, Booth NA, Mutch NJ. The antifibrinolytic function of factor XIII is exclusively expressed through a2-antiplasmin crosslinking. Blood. 2011;117:6371-4.
43. Sakata Y, Aoki N. Cross-linking of alpha2-plasmin inhibitor to fibrin by fibrin-stabilizing factor. J Clin Invest. 1980;65:290-7.
44. Koseki-Kuno S, Yamakawa M, Dickneite G, Ichinose A. Factor XIII A subunit-deficient mice developed severe uterine bleeding events and subsequent spontaneous miscarriages. Blood. 2003; 102:4410-2.
45. Souri M, Koseki-Kuno S, Takeda N, Yamakawa M, Takeishi Y, Degen JL, et al. Male-specific cardiac pathologies in mice lacking either the A or B subunit of factor XIII. Thromb Haemost. 2008; 99:401-8.
46. Lovejoy AE, Reynolds TC, Visich JE, Butine MD, Young G, Belvedere MA, Blain RC, Pederson SM, Ishak LM, Nugent DJ. Safety and pharmacokinetics of recombinant factor XIII-A2 administration in patients with congenital factor XIII deficiency. Blood. 2006;108:57-62.
47. Asahina T, Kobayashi T, Takeuchi K, Kanayama N. Congenital blood coagulation factor XIII deficiency and successful deliveries: a review of the literature. Obstet Gynecol Surv. 2007;62: 255-60.
48. Girolami A, Burul A, Sticchi A. Congenital deficiency of factor XIII with normal subunit S and lack of subunit A. Report of a new family. Acta Haematol. 1977;58:17-26.
49. Izumi T, Hashiguchi T, Castaman G, Tosetto A, Rodeghiero F, Girolami A, Ichinose A. Type I factor XIII deficiency is caused by a genetic defect of its b subunit: insertion of triplet AAC in exon III leads to premature termination in the second Sushi domain. Blood. 1996;87:2769-74.
50. Saito M, Asakura H, Yoshida T, Ito K, Okafuji K, Yoshida T, Matsuda T. A familial factor XIII subunit B deficiency. Br J Haematol. 1990;74:290-4.
51. Capellato MG, Lazzaro AR, Marafioti F, Polato G, Girolami A. A new family with congenital factor XIII deficiency showing a deficit of both subunit A and B. Type I factor XIII deficiency. Haematologia (Budap). 1987;20:179-87.
52. Hashiguchi T, Saito M, Morishita E, Matsuda T, Ichinose A. Two genetic defects in a patient with complete deficiency of the b-subunit for coagulation factor XIII. Blood. 1993;82:145-50.
53. Souri M, Izumi T, Higashi Y, Girolami A, Ichinose A. A founder effect is proposed for factor XIII B subunit deficiency caused by the insertion of triplet AAC in exon III encoding the second Sushi domain. Thromb Haemost. 1998;80:211-3.
54. Alvarado LR, Lovejoy AE, Nakagawa P, Hsieh LB, Chediak J, Williams SA, Nugent DJ. A novel mutation in exon 10 of factor XIII subunit B. J Thromb Haemost. 2007;5(SUPPL. 2):P-T-043.