The biology of prothrombin

Critical Reviews in Eukaryotic Gene Expression

Volumn 8, Issue 2, 1998, Pages 203-224

  Degen, Sandra J F ^a,b   Sun, William Y ^a  

^a UNIVERSITY OF CINCINNATI   (United States)

^b CINCINNATI CHILDREN S HOSPITAL RESEARCH FOUNDATION   (United States)

Author keywords

Blood coagulation; Gene targeting; HNF 1; Kringle domains; Prothrombin deficiency; Vitamin K dependent protein

Indexed keywords

PROTHROMBIN;

BLEEDING; EMBRYO DEATH; EMBRYO DEVELOPMENT; GENE CONTROL; GENE EXPRESSION; GENE STRUCTURE; HUMAN; LIVER; NONHUMAN; PRIORITY JOURNAL; PROTHROMBIN DEFICIENCY; REVIEW; TISSUE DISTRIBUTION;

EID: 0032470465     PISSN: 10454403     EISSN: None     Source Type: Journal    
DOI: 10.1615/CritRevEukarGeneExpr.v8.i2.60     Document Type: Review

References (98)

1. Arni RK, Padmanabhan K, Padmanabhan KP, Wu T-P, Tulinsky, A (1993): Structures of the noncovalent complexes of human and bovine prothrombin fragment 2 with human PPACK- thrombin. Biochemistry 32:4727-4737.
2. Bachhuber BG, Sarembock IJ, Gimple LW, McNamara CA, Owens GK (1995): Thrombin-induced mitogenesis in cultured aortic smooth muscle cells requires prolonged thrombin exposure. Am J Pathol 286:C1141-C1147.
3. Bajzar L, Manuel R, Nesheim ME. (1995): Purification and characterization of TAFI, a thrombin-activatable fibrinolysis inhibitor. J Biol Chem 270:14477-14484.
4. Bancroft JD, Schaefer LA, Degen SJF (1990): Characterization of the Alu-rich 5′-flanking region of the human prothrombin-encoding gene: identification of a positive cis-acting element that regulates liver-specific expression. Gene 95:253-260.
5. Bancroft JD, McDowell SA, Degen SJF (1992): The human prothrombin gene: transcriptional regulation in HepG2 cells. Biochemistry 31, 12469-12476.
6. Banfield DK, MacGillivray RTA (1992): Partial characterization of vertebrate prothrombin cDNAs: amplification and sequence analysis of the B chain of thrombin from nine different species. Proc Natl Acad Sci USA 89:2779-2783.
7. Bar-Shavit R, Kahn A, Mann KG, Wilner GD (1986a): Identification of a thrombin sequence with growth factor activity on macrophages. Proc Natl Acad Sci USA 83:976-980.
8. Bar-Shavit R, Hruska KA, Kahn AJ, Wilner GD (1986b): Hormone-like activity of human thrombin. Ann New York Acad Sci 485:335-348.
9. Bar-Shavit R, Hruska KA, Kahn AJ Wilner GD (1987): Thrombin chemotactic stimulation of HL-60 cells: studies on thrombin responsiveness as a function of differentiation. J Cell Physiol 131:255-161.
10. Bar-Shavit R, Eldor A, Vlodavsky, I (1989): Binding of thrombin to subendothelial extracelluar matrix: protection and expression of functional properties. J Clin Invest 84:1096-1104.
11. Bar-Shavit R, Benezra M, Eldor A, Hy-am E, Fenton JW 2d, Wilner GD, Vlodavasky I (1990): Thrombin immobilized to extracellular matrix is a potent mitogen for vascular smooth muscle cells: nonenzymatic mode of actin. Cell Reg 1:453-463.
12. Barnhart MI (1960): Cellular site for prothrombin synthesis. Am J Physiol 199:360-366.
13. Bi L, Lawler AM, Antonarakis SE, High A, Gearhart JD, Kazazian HH Jr, (1995): Targeted disruption of the mouse factor VIII gene produces a model of haemophilia A. Nature Genetics 10:119-121.
14. Bugge TH, Flick MJ, Daugherty CC, Degen JL (1995): Plasmingen deficiency causes severe thrombosis but is compatible with development and reproduction. Genes Dev 9:794-807.
15. Bugge TH, Suh TT, Flick MJ, Daugherty CC, Romer J, Solberg H, Ellis V, Dano K, Degen JL (1995): The receptor for urokinase-type plasminogen activator is not essential for mouse development or fertility. J Biol Chem 270:16886-16894.
16. Bugge TH, Xiao Q, Kombrinck KW, Flick MJ, Holmback K, Danton MJS, Colbert MC, Witte DP, Fujikawa K, Davie EW, Degen JL (1996): Fatal embryonic bleeding events in mice lacking tissue factor, the cell-associated initiator of blood coagulation. Proc Natl Acad Sci USA 93:6258-6263.
17. Butkowski RJ, Elion J, Downing MR, Mann KG (1977): Primary structure of human prethrombin 2 and α-thrombin. J Biol Chem 252:4942-4957.
18. Carmeliet P, Schoonjans L, Kieckens L, Ream B, Degen J, Bronson R, De Vos, R, van den Oord JJ, Collen D, Mulligan RC (1994): Physiological consequences of loss of plasminogen activator gene function in mice. Nature 368:419-424.
19. Carmeliet P, Mackman N, Moons L, Luther T, Gressens P, Vlaenderen IV, Demunch H, Kasper M, Breier G, Evrard P, Muller M, Risau W, Edgington T, Collen D (1996): Role of tissue factor in embryonic blood vessel development. Nature 383:73-75.
20. Cavanaugh KP, Gurwitz D, Cunningham DD, Bradshaw RA (1990): Reciprocal modulation of astrocyte stellation by thrombin and protease nexin-1. J Neurochem 54:1735-1743.
21. Chen LB, Buchanan JM (1975): Mitogenic activity of blood components. I. Thrombin and prothrombin. Proc Natl Acad Sci USA 72:131-135.
22. Chow BK-C, Ting V, Tufaro F, MacGillivray RTA (1991): Characterization of a novel liver- specific enhancer in the human prothrombin gene. J Biol Chem 266:18927-18933.
23. Connolly AJ, Ishihara H, Kahn ML, Farese RV Jr, Coughlin SR (1996): Role of the thrombin receptor in development and evidence for a second receptor. Nature 381:516-519.
24. Corrigan JJ, Jeter M, Earnest DL (1982): Prothrombin antigen and coagulant activity in patients with liver disease. J Am Med Assoc 248:1736-1739.
25. Courtois G, Baumhueter S, Crabtree GR (1988): Purified hepatocyte nuclear factor 1 interacts with a family of hepatocyte-specific promoters. Proc Natl Acad Sci USA 85:7937-7994.
26. Cui J, O'Shea KS, Purkayastha A, Saunders TL, Ginsburg D (1996): Fatal haemorrhage and incomplete block to embryogenesis in mice lacking coagulation factor V. Nature 384:66-68.
27. Cunningham DD, Farrell DH (1986): Thrombin interactions with cultured fibroblasts: relationship to mitogenic stimulation. Ann NY Acad Sci 485:240-248.
28. Darrow AL, Fung-Leung W-P, Ye RD, Santulli RJ, Cheung W-M, Derian CK, Burns CL, Damiano BP, Zhou L, Keenan CM, Peterson PA, Andrade-Gordon P (1996): Biological consequences of thrombin receptor deficiency in mice. Throm Haem 76:860-866.
29. Davey MG, Luscher EF (1967): Actions of thrombin and other coagulant and proteolytic enzymes on blood platelets. Nature 216:857-858.
30. Davie EW, Fujikawa K, Kisiel W (1991): The coagulation cascade: initiation, maintenance, and regulation. Biochemistry 30:10363-10370.
31. Degen SJF, MacGillivray RTA, Davie EW (1983): Characterization of the complementary deoxyribonucleic acid and gene coding for human prothrombin. Biochemistry 22:2087-2097.
32. Degen SJF, Davie EW (1987): Nucleotide sequence of the gene for human prothrombin. Biochemistry 26:6165-6177.
33. Degen SJF, Schaefer LA, Jamison CS, Grant SG, Fitzgibbon JJ, Pai J-A, Chapman VM, Elliott RW (1990): Characterization of the cDNA coding for mouse prothrombin and localization of the gene on mouse chromosome 2. DNA Cell Biol 9:487-498.
34. Degen SJF (1992): The prothrombin gene and its liver-specific expression. Semin Thromb Hemost 18:230-242.
35. Degen SJF (1995): Prothrombin. In Roberts H, High K (eds): "Molecular Biology of Thrombosis and Hemostasis" New York: Marcel Dekker, 75-99.
36. Dihanich M, Monard D (1990): cDNA sequence of rat prothrombin. Nucleic Acids Res 18:4251.
37. Dihanich M, Kaser M, Reinhard E, Cunningham D, Monard D (1991): Prothrombin mRNA is expressed by cells of the nervous system. Neuron 6:575-581.
38. DiScipio RG, Hermodson MA, Yates SG, Davie EW (1977): A comparison of human prothrombin, factor IX (Christmas factor), factor X (Stuart factor), and protein S. Biochemistry 16:698-706.
39. DiSimone V, Cortese R (1992): Transcription factors and liver-specific genes. Biochim Biophys Acta 1132:119-126.
40. Esmon CT, Jackson CM (1974): The conversion of prothrombin to thrombin. IV. The function of the fragment 2 region during activation in the presence of factor V. J Biol Chem 249:7791-7797.
41. Esmon CT (1983): Protein C: Biochemistry, physiology, and clinical implications. Blood 62:1155-1158.
42. Fair DS, Bahnak BR (1984): Human hepatoma cells secrete single chain factor X, prothrombin, and antithrombin III. Blood 64:194-204.
43. Fenton JW, Olson TA, Zabinski MP, Wilner GD (1988): Anion-binding exosite of human α-thrombin and fibrin(ogen) recognition. Biochemistry 27:7106-7112.
44. Furie B, Furie BC (1992): Molecular and cellular biology of blood coagulation. N Eng J Med 326:800-806.
45. Grabham P, Cunningham DD (1995): Thrombin receptor activation stimulates astrocyte proliferation and reversal of stellation by distinct proliferation and reversal of stellation by distinct pathways: involvement of tyrosine phosphorylation. J Neurochem 64:583-591.
46. Gurwitz D, Cunningham DD (1988): Thrombin modulates and reverses neuroblastoma neurite outgrowth. Proc Natl Acad Sci USA 85:3440-3444.
47. Healy AM, Rayburn HB, Rosenberg RD, Weiler H (1995): Absence of the blood-clotting regulator thrombomodulin causes embryonic lethality in mice before development of a functional cardiovascular system. Proc Natl Acad Sci USA 92:850-854.
48. Huang Z-F, Higuchi D, Lasky N, Broze Jr GJ (1997): Tissue factor pathway inhibitor gene disruption produces intrauterine lethality in mice. Blood 90:944-951.
49. Irwin DM, Robertson KA, MacGillivray RTA (1988): Structure and evolution of the bovine prothrombin gene. J Mol Biol 200:31-45.
50. Ishihara H, Connolly AJ, Zeng D, Kahn ML, Zheng YW, Timmons C, Tram T, Coughlin SR (1997): Protease-activated receptor 3 is a second thrombin receptor in humans. Nature 386:502-506.
51. Jamison CS, Degen SJF (1991): Prenatal and postnatal expression of mRNA coding for rat prothrombin. Biochim Biophys Acta 1088:208-216.
52. Karpatkin M, Tang Z, Meer J, Blei F, Samuels H (1991): Cloning and partial sequence of a cDNA for rabbit prothrombin. Thromb Res 62:757-763.
53. Kotkow KJ, Deitcher SR, Furie B, Furie BC (1995): The second kringle domain of prothrombin promotes Factor Va-mediated prothrombin activation by prothrombinase. J Biol Chem 270:4551-4557.
54. Krayev AS, Markusheva TV, Kramerov DA, Ryskov AP, Skryabin KG, Bayev AA. Georgiev GP (1982): Ubiquitous transposon-like repeats B1 and B2 of the mouse genome: B2 sequencing. Nucl Acids Res 10:7461-7475.
55. Lin H-F. Maeda N, Smithies O, Straight DL, Stafford DW (1997): A coagulation factor IX-deficient mouse model for human hemophilia B. Blood 90:3962-3966.
56. Liotta LA, Goldfarb RH, Brundage R, Siegal GP, Terranova V, Garbisa S (1981): Effect of plasminogen activator (urokinase), plasmin and thrombin on glycoprotein and collagenous components of basement membrane. Cancer Res 41:4629-4636.
57. Liu L-W, Vu T-KH, Esmon CT, Coughlin SR (1991): The region of the thrombin receptor resembling hirudin binds to thrombin and alters enzyme specificity. J Biol Chem 266:16977-16980.
58. MacGillivray RTA, Degen SJF, ChandraT, Woo SLC, Davie EW (1980): Cloning and analysis of a cDNA coding for bovine prothrombin. Proc Natl Acad Sci USA 77:5153-5157.
59. MacGillivray RTA, Davie EW (1984): Characterization of bovine prothrombin mRNA and its translation product. Biochemistry 23:1626-1634.
60. Magnusson S, Petersen TE, Sottrup-Jensen L, Claeys H (1975): Complete primary structure of prothrombin: isolation, structure, and reactivity of ten carboxylated glutamic acid residues and regulation of prothrombin activation by thrombin. In Reich E, Rifkin DB, Shaw E (eds): "Proteases and Biological Control" Cold Spring Harbor, NY: Cold Spring Harbor Laboratories, 123-149.
61. Maniatis T, Goodbourn S, Fischer JA (1987): Regulation of inducible and tissue-specific gene expression. Science 236:1237-1245.
62. Mann KG, Elion J (1980): Prothrombin. In Schmidt RM (ed) "CRC Handbook Series in Clinical Laboratory Science, Section I: Hematology" Boca Raton, FL: CRC Press vol. 3:15-31.
63. Markwardt F (1970): Hirudin as an inhibitor of thrombin. Methods Enzymol 19:924-932.
64. Mitchell PJ, Tjian R (1989): Transcriptional regulation in mammalian cells by sequence-specific DNA binding proteins. Science 245:371-378.
65. Nelken NA, Soilfer SJ, O'Keefe J, Vu TK, Charo IF, Coughlin SR (1992): Thrombin receptor expression in normal and atherosclerotic human arteries. J Clin Invest 90:1614-1621.
66. Nelsestuen GL, Zytkovicz TH, Howard JB (1974): A mode of action of vitamin K: identification of γ-carboxy-glutamic acid as a component of prothrombin. J Biol Chem 249:6347-6350.
67. Neveu I, Jehan F, Jandrot-Perrus M, Wion D, Brachet P (1993): Enhancement of the synthesis and secretion of nerve growth factor in primary cultures of glial cells by proteases: a possible involvement of thrombin. J Neurochem 60:858-867.
68. Nishino A, Suzuki M, Yoshimoto T, Otani H, Nagura H (1994): A novel aspect of thrombin in the tissue reaction following central nervous system injury. Acta Neurochirurgica (suppl) 60:86-88.
69. Noe G, Hofsteenge J, Rovelli G, Stone SR (1988): The use of sequence-specific antibodies to identify a secondary binding site in thrombin. J Biol Chem 263:11729-11735.
70. Patthy L, Trexler M, Vali Z, Banyai L, Varadi A (1984): Modules specialized for protein binding. Homology of the gelatin-binding region of fibronectin with the kringle structures of proteases. FEBS Lett 171:131-136.
71. Patthy L (1985): Evolution of the proteases of blood coagulation and fibrinolysis by assembly from modules. Cell 41:657-663.
72. Romer J, Bugge TH, Pyke C, Lund LR, Flick MJ, Degen JL, Dano K (1996): Impaired wound healing in plasminogen-deficient mice. Nature Med 2:287-292.
73. Rosen ED, Chan JCY, Idusogie E, Clotman F, Vlasuk G, Luther T, Jalbert LR, Albrecht S, Zhong L, Lissens A, Schoonjans L, Moons L, Collen D, Castellino FJ, Carmeliet P (1997): Mice lacking factor VII develop normally but suffer fatal perinatal bleeding. Nature 390:290-294.
74. Royle NJ, Irwin DM, Koschinsky ML, MacGillivray RTA, Hamerton JL (1987): Human genes encoding prothrombin and ceruloplasmin map to 11p11-q12 and 3q21-24, respectively. Som Cell Mol Genet 13:285-292.
75. Rydel TJ, Ravichandran KG, Tulinsky A, Bode W, Huber R, Roitsch C, Fenton JW II (1990): The structure of a complex of recombinant hirudin and human a-thrombin. Science 249:277-280.
76. Schmid CW, Jelinek WR (1982): The Alu family of dispersed repetitive sequences. Science 216:1065-1070.
77. Seegers H (1979): Prothrombin yesterday and today. Prog Chem Fibrinolysis Thrombolysis 4:241-254.
78. Sheehan JP, Wu Q, Tollefsen DM, Sadler JE (1993): Mutagenesis of thrombin selectively modulates inhibition by seripins heparin cofactor II and antithrombin III: interaction with the anion-binding exosite determined heparin cofactor II specificity. J Biol Chem 268:3639-3645.
79. Shivdasani RA, Rosenblatt MF, Zucker-Franklin D, Jackson CW, Hunt P, Saris CI, Orkin SH (1995): Transcription factor NF-E2 is required for platelet formation independent of the actions of thrombopoietin/MGDF in megakaryocyte development. Cell 81:695-704.
80. Shivdasani RA, Fielder P, Keller G-A, Orkin SH, de Sauvage FJ (1997): Regulation of the serum concentration of thrombopoietin in thrombocytopenic NF-E2 knockout mice. Blood 90:1821-1827.
81. Soifer SJ, Peters KG, O'Keefe J, Coughlin SR (1994): Disparate temporal expression of the prothrombin and thrombin receptor genes during mouse development. Am J Pathol 144:60-69.
82. Stenflo J, Fernlund P, Egan W, Roepstorff P (1974): Vitamin K-dependent modifications of glutamic acid residues in prothrombin. Proc Natl Acad Sci USA 71:2730-2733.
83. Stenflo J, Suttie JW (1977): Vitamin K-dependent formation of γ-carboxyglutamic acid. Ann Rev Biochem 46:157-172.
84. Suh TT, Holmback K, Jensen NJ, Daugherty CC, Small K, Simon DI, Potter SS, Degen JL (1995): Resolution of spontaneous bleeding events but failure of pregnancy in fibrinogen-deficient mice. Genes Dev 9:2020-2033.
85. Sun WY, Witte DP, Degen JL, Colbert MC, Burkart MC, Holmback K, Xiao Q, Bugge TH, Degen SJF (1998): Prothrombin deficiency results in embryonic and neonatal lethality in mice. Proc Natl Acad Sci USA 95:7597-7602.
86. Thompson AR, Enfield DL, Ericsson LH, Legaz ME, Fenton JW II (1977): Human thrombin: partial primary structure. Arch Biochem Biophys 178:356-367.
87. Toomey JR, Kratzer KE, Lasky N, Stanton JJ, Broze GJ Jr (1996): Targeted disruption of the murine tissue factor gene results in embryonic lethality. Blood 88:1583-1587.
88. Toomey JR, Kratzer KE, Lasky NM, Broze GJ Jr (1997): Effect of tissue factor deficiency on mouse and tumor development. Proc Natl Acad Sci USA 94:6922-6926.
89. Trexler M, Patthy L (1983): Folding autonomy of the kringle 4 fragment of human plasminogen. Proc Natl Acad Sci USA 80:2457-2461.
90. Tsiang M, Lentz SR, Dittman WA, Wen D, Scarpati EM, Sadler JE (1990): Equilibrium binding of thrombin to recombinant human thrombomodulin: effect of hirudin, fibrinogen, factor Va and peptide analougues. Biochemistry 29:10602-10612.
91. Tulinsky A, Park CH, Skrzypczak-Jankun E (1988): Structure of prothrombin fragment 1 refined at 2.8 A resolution. J Mol Biol 20:885-901.
92. van Zonneveld A-J, Veerman H, Pannekoek H (1986): Autononomous functions of structural domains on human tissue-type plasminogen activator. Proc Natl Acad Sci USA 83:4670-4674.
93. Vaughan PJ, Pike CJ, Cotman CW, Cunningham D (1995): Thrombin receptor activation protects neurons and astrocytes from cell death produced by environmental insults. J Neurosci 15:5389-5401.
94. Vu T-KH, Hung DT, Wheaton VI, Coughlin SR (1991): Molecular cloning of a functional thrombin receptor reveals a novel proteolytic mechanism of receptor activation. Cell 64:1057-1068.
95. Walz DA, Hewett-Emmett D, Seegers WH (1977): Amino acid sequence of human prothrombin fragments 1 and 2. Proc Natl Acad Sci USA 74:1969-1972.
96. Weinstein JR, Gold SJ, Cunningham DD, Gall CM (1995): Cellular localization of thrombin receptor mRNA in rat brain: expression by mesencephalic dopaminergic neurons and codistribution with prothrombin mRNA. J Neurosci 15:2906-2919.
97. Wu Q, Tsiang M, Sadler JE (1992): Localization of the single-stranded DNA binding site in the thrombin anion-binding exosite. J Biol Chem 267:24408-24412.
98. Xue J, Wu Z, Westfield LA, Tuley EA, Lu D, Zhang Q, Shim K, Zheng X, Sadler JE (1998): Incomplete embryonic lethality and fatal neonatal hemorrhage caused by prothrombin deficiency in mice. Proc Natl Acad Sci USA 95:7603-7607.