The Protein C Pathway: New Insights

• References

• 1 Esmon CT. The roles of protein C and thrombomodulin in the regulation of blood coagulation. J Biol Chem 1989; 264: 4743-4746
• 2 Dreyfus M, Magny JF, Bridey F, Schwarz HP, Planché C, Dehan M, Tchernia G. Treatment of homozygous protein C deficiency and neonatal purpura fulminans with a purified protein C concentrate. N Engl J Med 1991; 325: 1565-1568
• 3 Dahlbäck B. Physiological anticoagulation. Resistance to activated protein C and venous thromboembolism. J Clin Invest 1994; 94: 923-927
• 4 Ohlin A-K, Marlar RA. The first mutation identified in the thrombomodulin gene in a 45-year-old man presenting with thromboembolic disease. Blood 1995; 85 (02) 330-336
• 5 Healy AM, Rayburn HB, Rosenberg RD, Weiler H. Absence of the blood-clotting regulator thrombomodulin causes embryonic lethality in mice before development of a functional cardiovascular system. Proc Natl Acad Sci (USA) 1995; 92: 850-854
• 6 Cortellaro M, Boschetti C, Cofrancesco E, Zanussi C, Catalano M, de Gaetano G, Gabrielli L, Lombardi B, Specchia G, Tavazzi L. et al The PLAT Study: Hemostatic function in relation to atherothrombotic ischemic events in vascular disease patients. Principal results. Arterioscl Thromb 1992; 12: 1063-1070
• 7 Gruber A, Griffin JH, Harker LA, Hanson SR. Inhibition of platelet-dependent thrombus formation by human activated protein C in a primate model. Blood 1989; 73: 639-642
• 8 Hanson SR, Griffin JH, Harker LA, Kelly AB, Esmon CT, Gruber A. Antithrombotic effects of thrombin-induced activation of endogenous protein C in primates. J Clin Invest 1993; 92: 2003-2012
• 9 De Stefano V, Mastrangelo S, Schwarz HP, Pola P, Flore R, Bizzi B, Leone G. Replacement therapy with a purified protein C concentrate during initiation of oral anticoagulation in severe protein C congenital deficiency. Thromb Haemostas 1993; 3: 247-249
• 10 Powars D, Larsen R, Johnson J, Hulbert T, Sun T, Patch MJ, Francis R, Chan L. Epidemic meningococcemia and purpura fulminans with induced protein C deficiency. Clin Infect Dis 1993; 17: 254-261
• 11 Taylor FB, Chang Jr A, Hinshaw LB, Esmon CT, Archer LT, Beller BK. A model for thrombin protection against endotoxin. Thromb Res 1984; 36: 177-185
• 12 Taylor FB, Chang Jr A, Esmon CT, D’Angelo A, Vigano-D’Angelo S, Blick KE. Protein C prevents the coagulopathic and lethal effects of E coli infusion in the baboon. J Clin Invest 1987; 79: 918-925
• 13 Taylor F, Chang A, Ferrell G, Mather T, Catlett R, Blick K, Esmon CT. C4b-binding protein exacerbates the host response to Escherichia coli. Blood 1991; 78: 357-363
• 14 Grey S, Hau H, Salem HH, Hancock WW. Selective effects of protein C on activation of human monocytes by lipopolysac-charide, interferon-gamma, or PMA: Modulation of effects on CD11b and CD14 but not CD25 or CD54 induction. Transplant Proc 1993; 25: 2913-2914
• 15 Grey ST, Hancock WW. A physiologic anti-inflammatory pathway based on thrombomodulin expression and generation of activated protein C by human mononuclear phagocytes. J Immunol 1996; 156: 2256-2263
• 16 Gerson WT, Dickerman JD, Bovill EG, Golden E. Severe acquired protein C deficiency in purpura fulminans associated with disseminated intravascular coagulation: treatment with protein C concentrate. Pediatrics 1993; 91: 418-422
• 17 Rivard GE, David M, Farrell C, Schwarz HP. Treatment of purpura fulminans in meningococcemia with protein C concentrate. J Pediatr 1995; 126 (04) 646-652
• 18 Rivard GE, David M, Farrell C, Gerson W, Dickerman JD, Bovill EG, Schwarz HP. Treatment of purpura fulminans in meningococcemia with protein C concentrate. [Abstract] Thromb Haemost 1993; 69: 2338
• 19 Esmon CT, Schwarz HP. An update on clinical and basic aspects of the protein C anticoagulant pathway. Trends Cardiovasc Med 1995; 5: 141-148
• 20 Coughlin SR. Thrombin receptor function and cardiovascular disease. Trends Cardiovasc Med 1994; 4: 77-83
• 21 Fukudome K, Esmon CT. Identification, cloning and regulation of a novel endothelial cell protein C/activated protein C receptor. J Biol Chem 1994; 269: 26486-26491
• 22 Bangalore N, Drohan WN, Orthner CL. High affinity binding sites for activated protein C and protein C on cultured human umbilical vein endothelial cells. Independent of protein S and distinct from known ligands. Thromb Haemostas 1994; 72 (03) 465-474
• 23 Fukudome K, Kurosawa S, Steams-Kurosawa DJ, He X, Rezaie AR, Esmon CT. The endothelial cell protein C receptor: Cell surface expression and direct ligand binding by the soluble receptor. J Biol Chem 1996; 271: 17491-17498
• 24 Esmon CT, Simionescu N, Simionescu M. editors. Endothelial cell biology. New York: Plenum Publishing, Corp; 1988. 09. Assembly and function of the protein C anticoagulant pathway on endothelium,p. 191-206
• 25 Esmon NL. Thrombomodulin. Sem Thromb Hemost 1987; 13: 454-463
• 26 Steams-Kurosawa DJ, Kurosawa S, Mollica JS, Ferrell GL, Esmon CT. The endothelial cell protein C receptor augments protein C activation by the thrombin-thrombomodulin complex. Proc Nati Acad Sci (USA) 1996; 93: 10212-10216
• 27 Mathews II, Padmanabhan KP, Tulinsky A. Structure of a nonadecapeptide of the fifth EGF domain of thrombomodulin complexed with thrombin. Biochemistry 1994; 33: 13547-13552
• 28 Stubbs MT, Oschkinat H, Mayr I, Huber R, Angliker H, Stone SR, Bode W. The interaction of thrombin with fibrinogen. A structural basis for its specificity. Eur J Biochem 1992; 206: 187-195
• 29 Rydel TJ, Ravichandran KG, Tulinsky A, Bode W, Huber R, Roitsch C, Fenton II JW. The structure of a complex of recombinant hirudin and human a-thrombin. Science 1990; 249: 277-280
• 30 Mathews II, Padmanabhan KP, Ganesh V, Tulinsky A, Ishii M, Chen J, Turck CW, Coughlin SR, Fenton JW. Crystallographic structures of thrombin complexed with thrombin receptor peptides: existence of expected and novel binding modes. Biochemistry 1994; 33: 3266-32679
• 31 Ye J, Esmon NL, Esmon CT, Johnson AE. The active site of thrombin is altered upon binding to thrombomodulin: Two distinct structural changes are detected by fluorescence, but only one correlates with protein C activation. J Biol Chem 1991; 266: 23016-23021
• 32 Mather T, Oganessyan V, Hof P, Huber R, Foundling S, Esmon C, Bode W. The 2.8 Å crystal structure of Gla-domainless activated protein CEMBO J. 1996; 15: 6822-6831
• 33 Brinkhous KM, Sigman JL, Read MS, Stewart PF, McCarthy KP, Timony GA, Leppanen SD, Rup B J, Keith JC, Garzone Jr PD. et al Recombinant human factor IX: Replacement therapy, prophylaxis, and pharmacokinetics in canine hemophilia B. Blood 1996; 88: 2603-2610
• 34 Cheung WF, van den Born J, Kuhn K, Kjellen L, Hudson BG, Stafford DW. Identification of the endothelial cell binding site for factor IX. Proc Natl Acad Sci (USA) 1996; 93: 11068-11073
• 35 Laszik Z, Esmon CT. The newly identified protein C receptor is present primarily on vascular endothelium of large vessels: Implications for the control of the protein C pathway. [In Press] J Clin Invest. 1997
• 36 Ding W, Gu JM, Fukudome K, Laszik Z, Grammas P, Esmon CT. Upregulation of the message for rodent endothelial cell protein C receptor (EPCR) by endotoxin and thrombin. [Abstract] Circulation 1996; 94: 1694