Nonenzymatic anticoagulant activity of the mutant serine protease Ser360Ala-activated protein C mediated by factor Va

Protein Science

Volumn 6, Issue 1, 1997, Pages 132-140

  Gale, Andrew J ^a   Sun, X I ^a,b   Heeb, Mary J ^a   Griffin, John H ^a  

^a SCRIPPS RESEARCH INSTITUTE   (United States)

^b Alliance Pharmaceutical Corporation   (United States)

Author keywords

anticoagulant; factor Va; protein C; protein protein interaction; serine protease

Indexed keywords

BLOOD CLOTTING FACTOR 5A; PROTEIN C; SERINE PROTEINASE;

ANTICOAGULATION; ARTICLE; ENZYME ACTIVATION; ENZYME ACTIVITY; ENZYME INACTIVATION; HUMAN; HUMAN CELL; HYDROLYSIS; KIDNEY CELL; PRIORITY JOURNAL; PROTEIN C DEFICIENCY; PROTEIN DEGRADATION; PROTEIN STRUCTURE; VEIN THROMBOSIS;

EID: 0031032080     PISSN: 09618368     EISSN: None     Source Type: Journal    
DOI: 10.1002/pro.5560060115     Document Type: Article

References (58)

1. Birktoft JJ, Blow DM. 1972. Structure of crystalline α-chymotrypsin (V). The atomic structure of tosyl-α-chymotrypsin at 2 Å resolution. J Mol Biol 68:187-240.
2. Branson HE, Katz J, Marble R, Griffin JH. 1983. Inherited protein C deficiency and a coumarin-responsive chronic relapsing purpura fulminans syndrome in a neonate. Lancet 2:1165-1168.
3. Carter P, Wells JA. 1988. Dissecting the catalytic triad of a serine protease. Nature 332:564-568.
4. Conard J, Bauer KA, Gruber A, Griffin JH, Schwarz HP, Horellou MH, Samama MM, Rosenberg RD. 1993. Normalization of markers of coagulation activation with a purified protein C concentrate in adults with homozygous protein C deficiency. Blood 82:1159-1164.
5. Corey DR, Craik CS. 1992. An investigation into the minimum requirements for peptide hydrolysis by mutation of the catalytic triad of trypsin. J Am Chem Soc 114:1784-1790.
6. Dreyfus M, Maghey JF, Bridey F, Schultz HP, Planche C, Dehan M, Tchernia G. 1991. Treatment of homozygous protein C deficiency and neonatal purpura fulminans with a purified protein C concentrate. N Engl J Med 325:1565-1568.
7. Dreyfus M, Masterson M, David M, Rivard GE, Müller F-M, Kreuz W, Beeg T, Minford A, Allgrove J, Cohen JD, Christoph J, Bergmann F, Mitchell VE, Haworth C, Nelson K, Schwarz HP. 1995. Replacement therapy with a monoclonal antibody purified protein C concentrate in newborns with severe congenital protein C deficiency. Semin Thromb Hemost 21:371-381.
8. Emerick SC, Bang NU, Yan SB, Long GL, Harms CS, Huss C, Marks CA, Mattler LE, Comp PC, Esmon NB, Esmon CT. 1985. Antithrombotic properties of activated human protein C (#1282). Blood 66:349a.
9. Esmon CT, Owen WG. 1981. Identification of an endothelial cell cofactor for thrombin-catalyzed activation of protein C. Proc Natl Acad Sci USA 78:2249-2252.
10. Esmon CT, Taylor FB Jr, Snow TR. 1991. Inflammation and coagulation: Linked processes potentially regulated through a common pathway mediated by protein C. Thromb Haemost 66:160-165.
11. España F, Gruber AG, Heeb MJ, Hanson SR, Harker LA, Griffin JH. 1991. In vivo and in vitro complexes of activated protein C with two inhibitors in baboon plasma. Blood 77:1754-1760.
12. Fay PJ, Beattie T, Huggins CF, Regan LM. 1994. Factor VIIIa A2 subunit residues 558-565 represent a factor IXa interactive site. J Biol Chem 269:20522-20527.
13. Fehlhammer H, Bode W, Huber R. 1977. Crystal structure of bovine trypsinogen at 1.8 Å resolution. II. Crystallographic refinement, refined crystal structure and comparison with bovine trypsin. J Mol Biol 111:415-438.
14. Freer ST, Kraut J, Robertus JD, Wright HT, Xuong NH. 1970. Chymotrypsinogen: 2.5-Å crystal structure, comparison with α-chymotrypsin, and implications for zymogen activation. Biochemistry 9:1997-2009.
15. Gettins P, Patston PA, Schapira M. 1992. Structure and mechanism of action of serpins. Hematol Oncol Clin North Am 6:1393-1408.
16. Gladson CL, Groncy P, Griffin JH. 1987. Coumarin necrosis, neonatal purpura fulminans and protein C deficiency. Arch Dermatol 123:1701-1706.
17. Grey ST, Tsuchida A, Hau H, Orthner CL, Salem HH, Hancock WW. 1994. Selective inhibitory effects of the anticoagulant activated protein C on the responses of human mononuclear phagocytes to LPS, IFN-gamma, or phorbol ester. J Immunol 153:3664-3672.
18. Griffin JH, Evatt B, Zimmerman TS, Kleiss AJ, Wideman C. 1981. Deficiency of protein C in congenital thrombotic disease. J Clin Invest 68:1370-1373.
19. Gruber A, Griffin JH, Harker L, Hanson SR. 1989. Inhibition of platelet-dependent thrombus formation by human activated protein C in a primate model. Blood 73:639-642.
20. Gruber A, Griffin JH. 1992. Direct detection of activated protein C in blood from human subjects. Blood 79:2340-2348.
21. Hartley BS. 1970. Homologies in serine proteinases. Philos Trans R Soc Lond [Biol] 257:77-87.
22. 358. J Biol Chem 265:2365-2369.
23. 1-antitrypsin. J Biol Chem 263:11613-11616.
24. 2-antiplasmin in blood and comparisons to inhibition of factor Xa, thrombin, and plasmin. J Biol Chem 226:17606-17612.
25. Heeb MJ, Kojima Y, Hackeng TM, Griffin JH. 1994. Functional studies of factor Va (FVa) residues 493-506 related to activated protein C (APC) resistance. Blood 84:391a.
26. 506-factor V. Blood 85:3405-3411.
27. Heeb MJ, Kojima Y, Hackeng TM, Griffin JH. 1996. Binding sites for blood coagulation factor Xa and protein S involving residues 493-506 in factor Va. Protein Sci 5:1883-1889.
28. Heeb MJ, Mesters RM, Tans G, Rosing J, Griffin JH. 1993. Binding of protein S to factor Va associated with inhibition of prothrombinase that is independent of activated protein C. J Biol Chem 268:2872-2877.
29. Heeb MJ, Schwarz HP, White T, Lämmle B, Berrettini M, Griffin JH. 1988. Immunoblotting studies of the molecular forms of protein C in plasma. Thromb Res 52:33-43.
30. Kalafatis M, Mann KG. 1993. Role of the membrane in the inactivation of factor Va by activated protein C. J Biol Chem 265:27246-27257.
31. Kalafatis M, Rand MD, Mann KG. 1994. The mechanism of inactivation of human factor V and human factor Va by activated protein C. J Biol Chem 269:31869-31880.
32. Kisiel W. 1979. Human plasma protein C. Isolation, characterization and mechanism of activation by α-thrombin. J Clin Invest 64:761-769.
33. Kisiel W, Canfield WM, Ericsson LH, Davie EW. 1977. Anticoagulant properties of bovine plasma protein C following activation by thrombin. Biochemistry 16:5824-5831.
34. Kossiakoff AA, Chambers JL, Kay LM, Stroud RM. 1977. Structure of bovine trypsinogen at 1.9 Å resolution. Biochemistry 16:654-664.
35. Kraut J. 1977. Serine proteases: Structure and mechanism of catalysis. Annu Rev Biochem 46:331-358.
36. Kunkel TA, Roberts JD, Zakour RA. 1987. Rapid and efficient site-specific mutagenesis without phenotypic selection. Methods Enzymol 154:367-382.
37. Liao D-I, Remington SJ. 1990. Structure of wheat serine carboxypeptidase II at 3.5-Å resolution. A new class of serine protcinase. J Biol Chem 265:6528-6531.
38. Marlar RA, Kleiss AJ, Griffin JH. 1982. Mechanism of action of human activated protein C, a thrombin-dependent anticoagulant enzyme. Blood 59:1067-1072.
39. Mesters RM, Houghten RA, Griffin JH. 1991. Identification of a sequence of human activated protein C (residues 390-404) essential for its anticoagulant activity. J Biol Chem 266:24514-24519.
40. Murakami K, Okajima K, Uchiba M, Johno M, Nakagaki T, Okabe H, Takatsuki K. 1996. Activated protein C attenuates endotoxin-induced pulmonary vascular injury by inhibiting activated leukocytes in rats. Blood 87:642-647.
41. O'Brien LM, Medved LV, Fay PJ. 1995. Localization of factor IXa and factor Villa interactive sites. J Biol Chem 270:27087-27092.
42. Okajima K, Koga S, Kaji M, Inoue M, Nakagaki T, Funatsu A, Okabe H, Takatsuki K, Aoki N. 1990. Effect of protein C and activated protein C on coagulation and fibrinolysis in normal human subjects. Thromb Haemost 63:48-53.
43. Pan Y, DeFay T, Gitschier J, Cohen FE. 1995. Proposed structure of the A domains of factor VIII by homology modelling. Nat Struct Biol 2:740-744.
44. Perona JJ, Craik CS. 1995. Structural basis of substrate specificity in the serine proteases. Protein Sci 4:337-360.
45. Pichler L, Schramm W, Ulrich W, Varadi K, Schwarz HP. 1995. Antinociceptive properties of protein C in a model of inflammatory hyperalgesia in rats. Thromb Haemost 73:252-255.
46. Richardson MA, Gerlitz B, Grinnell BW. 1992. Enhancing protein C interactions with thrombin results in a clot-activated anticoagulant. Nature 360:261-264.
47. Rivard GE, David M, Farrell C, Schwarz HP. 1995. Treatment of purpura fulminans in meningococcemia with protein C concentrate. J Pediatr 126:646-652.
48. Schramm W, Spannagl M, Bauer KA, Rosenberg RD, Birkner B, Linnau Y, Schwarz HP. 1993. Treatment of coumarin-induced skin necrosis with a monoclonal antibody purified protein C concentrate. Arch Dermatol 129:753-756.
49. Seligsohn U, Berger A, Abead M, Rubin L, Attias D, Zivelin A, Rapaport SI. 1984. Homozygous protein C deficiency manifested by massive venous thrombosis in the newborn. N Engl J Med 310:559-562.
50. Snow TR, Deal MT, Dickey DT, Esmon CT. 1991. Protein C activation following coronary artery occlusion in the in situ porcine heart. Circulation 84:293-299.
51. Stenflo JA. 1976. A new vitamin K-dependent protein: Purification from bovine plasma and preliminary characterization. J Biol Chem 251:355-363.
52. Stroud RM, Kay LM, Dickerson RE. 1974. The structure of bovine trypsin: Electron density maps of the inhibited enzyme at 5 Å and at 2.7 Å resolution. J Mol Biol 83:185-208.
53. Taylor FB, Chang A, Esmon CT, D'Angelo A, Vigano-D'Angelo S, Blick KE. 1987. Protein C prevents the coagulopathic and lethal effects of Escherichia coli infusion in the baboon. J Clin Invest 79:918-925.
54. Tsuchida A, Salem H, Thomson N, Hancock WW. 1992. Tumor necrosis factor production during human renal allograft rejection is associated with depression of plasma protein C and free protein S levels and decreased intragraft thrombomodulin expression. J Exp Med 175:81-90.
55. Viganò S, Mannucci PM, Solinas S, Bottasso B, D'Angelo A, Mariani G. 1993. Early fall of protein C during short-term anticoagulant treatment. Thromb Haemost 50:310.
56. Walker FJ, Sexton PW, Esmon CT. 1979. The inhibition of blood coagulation by activated protein C through the selective inactivation of activated factor V. Biochim Biophys Acta 571:333-342.
57. Wang D, Bode W, Huber R. 1985. Bovine chymotrypsinogen A. X-ray crystal structure analysis and refinement of a new crystal form at 1.8 Å resolution. J Mol Biol 185:595-624.
58. 7D) of human activated protein C displays greatly reduced activity as an anticoagulant. Biochemistry 29:10828-10834.