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Thromb Haemost 1989; 61(03): 337-342
DOI: 10.1055/s-0038-1646591
Review Article
Schattauer GmbH Stuttgart

Protein C Inhibitor: Structure and Function

Koji Suzuki
1   The Department of Laboratory Medicine, Mie University School of Medicine, Tsu City, Mie
,
Yoshihiro Deyashiki
1   The Department of Laboratory Medicine, Mie University School of Medicine, Tsu City, Mie
,
Junji Nishioka
1   The Department of Laboratory Medicine, Mie University School of Medicine, Tsu City, Mie
,
Kazunori Toma
2   The Computer Science Department, Asahi Chemical Industry Fuji, Shizuoka, Japan
› Author Affiliations
Further Information

Publication History

Received 22 November 1988

Accepted after revision 30 January 1989

Publication Date:
24 July 2018 (online)

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Keywords

Protein C inhibit - Protein C pathway - Serine protease inhibitor - Serpin superfamily
 

  • References

  • 1 Rosenberg RD, Rosenberg JS. Natural anticoagulant mechanisms. J Clin Invest 1984; 74: 1-6
    CrossrefPubMedGoogle Scholar
  • 2 Stenflo J. A new vitamin K-dependent protein: Purification from bovine plasma and preliminary characterization. J Biol Chem 1976; 251: 355-363
    PubMedGoogle Scholar
  • 3 Esmon CT. Protein C: Biochemistry, physiology, and clinical implications. Blood 1983; 62: 1155-1158
    PubMedGoogle Scholar
  • 4 Suzuki K, Stenflo J, Dahlbäck B, Teodorsson B. Inactivation of human coagulation Factor V by activated protein C. J Biol Chem 1983; 258: 1914-1920
    PubMedGoogle Scholar
  • 5 Eaton D, Rodriguez H, Vehar GA. Proteolytic processing of human Factor VIII. Correlation of activated protein C with activation and inactivation of Factor VIII coagulant activity. Biochemistry 1986; 25: 505-512
    CrossrefPubMedGoogle Scholar
  • 6 Griffin JH. Clinical studies of protein C. Semin Thromb Hemostas 1984; 10: 161-162
    PubMedGoogle Scholar
  • 7 Comp PC, Nixon RR, Cooper MR, Esmon CT. Familial protein S deficiency is associated with recurrent thrombosis. J Clin Invest 1984; 74: 2082-2088
    CrossrefPubMedGoogle Scholar
  • 8 Kamiya T, Sugihara T, Ogata K, Saito H, Suzuki K, Nishioka J, Hashimoto S, Yamagata K. Inherited deficiency of protein S in a Japanese family with recurrent venous thrombosis: A study of three generations. Blood 1986; 67: 406-410
    PubMedGoogle Scholar
  • 9 Marlar RA, Griffin JH. Deficiency of protein C inhibitor in combined factor V/VIII deficiency disease. J Clin Invest 1980; 66: 1186-1189
    CrossrefPubMedGoogle Scholar
  • 10 Suzuki K, Nishioka J, Hashimoto S. Protein C inhibitor. Purification from human plasma and characterization J Biol Chem 1983; 258: 163-168
    PubMedGoogle Scholar
  • 11 Kusumoto K, Suzuki K, Hashimoto S. Purification and characterization of bovine protein C inhibitor: Comparison with a human protein. J Jpn Biochem Soc 1986; 58: 613 (abstr)
    PubMedGoogle Scholar
  • 12 Suzuki K, Nishioka J, Kusumoto H, Hashimoto S. Mechanism of inhibition of activated protein C by protein C inhibitor. J Biochem 1984; 95: 187-195
    CrossrefPubMedGoogle Scholar
  • 13 Meijjers JC M, Kanters DH A, Vlooswijk RA A, van Erp HE, Hessing M, Bouma BN. Inactivation of human plasma kallikrein and Factor XIa by protein C inhibitor. Biochemistry 1988; 27: 4231-4237
    CrossrefPubMedGoogle Scholar
  • 14 Stief TW, Radtke K-P, Heimburger N. Inhibition of urokinase by protein C inhibitor (PCI) – Evidence for identity of PCI and plasminogen activator inhibitor 3. Biol Chem Hoppe-Seyler 1987; 368: 1427-1433
    PubMedGoogle Scholar
  • 15 Heeb MJ, Espana F, Geiger M, Collen D, Stump DC, Griffin JH. Immunological identity of heparin-dependent plasma and urinary protein C inhibitor and plasminogen activator inhibitor-3. J Biol Chem 1987; 263: 15813-15816
    PubMedGoogle Scholar
  • 16 Morito F, Saito F, Suzuki K, Nishioka J. Synthesis and secretion of a cDNA for human protein C inhibitor by hepatoma-derived cell line, HepG2. Biochem Biophys Acta 1985; 884: 209-214
    PubMedGoogle Scholar
  • 17 Suzuki K. Biochemistry and physiology of protein C inhibitor. In: Protein C – Biochemical and Medical Aspects. Witt I. (ed.) Walter de Gruyter; Berlin: 1985. pp 43-58
    Google Scholar
  • 18 Suzuki K. Activated protein C inhibitor. Semin Thromb Hemostas 1984; 10: 154-161
    Article in Thieme ConnectPubMedGoogle Scholar
  • 19 Francis RB, Thomas W. Behavior of protein C inhibitor in intravascular coagulation and liver disease. Thromb Haemostas 1984; 52: 71-74
    PubMedGoogle Scholar
  • 20 Giddings JC, Sugrue A, Bloom AL. Quantitation of coagulant antigens and inhibition of activated protein C in combined factor V/ VII deficiency. Br J Haematol 1982; 52: 495-502
    CrossrefPubMedGoogle Scholar
  • 21 Canfield WM, Kiesiel W. Evidence of normal functional levels of activated protein C inhibitor in combined factor V/VIII deficiency disease. J Clin Invest 1982; 70: 1260-1272
    CrossrefPubMedGoogle Scholar
  • 22 Suzuki K, Nishioka J, Hashimoto S, Kamiya T, Saito H. Normal titer of functional and immunoreactive protein C inhibitor in plasma of patients with congenital combined deficiency of factor V and factor VIII. Blood 1983; 62: 1266-1270
    PubMedGoogle Scholar
  • 23 Marlar RA, Endres-Brooks J, Miller C. Serial studies of protein C and its plasma inhibitor in patients with disseminated intravascular coagulation. Blood 1985; 66: 59-65
    PubMedGoogle Scholar
  • 24 Nishioka J, Suzuki K. Determination of a complex formed with activated protein C and protein C inhibitor (CIC) and its clinical significance. Acta Haematol Jpn 1989; 52: 485 (Abstr)
    PubMedGoogle Scholar
  • 25 Van der Meer FJ M, van Tilburg NH, van der Linden IK, Briët E, Bertina RM. A second plasma inhibitor of activated protein C (APC). Thromb Haemostas 1987; 58: 277 (Abstr)
    PubMedGoogle Scholar
  • 26 Heeb MJ, Griffin JH. Physiologic inhibition of human activated protein C by α1-antitrypsin. J Biol Chem 1988; 263: 11613-11616
    PubMedGoogle Scholar
  • 27 Meijers JC M, Vlooswijk RA A, Kanters DH A J, Hessing M, Bouma BN. Identification of monoclonal antibodies that inhibit the function of protein C inhibitor. Evidence for heparin-independent inhibition of activated protein C in plasma. Blood 1988; 72: 1401-1403
    PubMedGoogle Scholar
  • 28 Börk I, Jackson CM, Jornvall H, Lavine KK, Nordling K, Salsgiver WJ. The active site of antithrombin III. Release of the same proteolytically cleaved form of the inhibitor from complexes with Factor IXa, Factor Xa, and thrombin. J Biol Chem 1982; 257: 2406-2411
    PubMedGoogle Scholar
  • 29 Suzuki K, Deyashiki Y, Nishioka J, Kurachi K, Akira M, Yamamoto S, Hashimoto S. Characterization of a cDNA for human protein C inhibitor. A new member of the plasma serine protease inhibitor superfamily J Biol Chem 1987; 262: 611-616
    PubMedGoogle Scholar
  • 30 Chandra T, Stackhouse R, Kidd VJ, Woo LC. Isolation and sequence characterization of a cDNA clone of human antithrombin III. Proc Natl Acad Sci USA 1983; 80: 1845-1848
    CrossrefPubMedGoogle Scholar
  • 31 Long GL, Chandra T, Woo SL, Davie EW, Kurachi K. Complete sequence of the cDNA for human α1-antitrypsin and the gene for the S variant. Biochemistry 1984; 23: 4828-4837
    CrossrefPubMedGoogle Scholar
  • 32 Chandra T, Stackhouse R, Kidd VJ, Robson KJ H, Woo SL C. Sequence homology between human α1-antichymotrypsin, α1-antitryp-sin, and antithrombin III. Biochemistry 1983; 22: 5055-5061
    CrossrefPubMedGoogle Scholar
  • 33 Ragg H. A new member of the plasma protease inhibitor gene family. Nucleic Acids Res 1986; 14: 1073-1088
    CrossrefPubMedGoogle Scholar
  • 34 Holmes WE, Nelles L, Lijnen HR, Collen D. Primary structure of human oq-antiplasmin, a serine protease inhibitor (serpin). J Biol Chem 1987; 262: 1659-1664
    PubMedGoogle Scholar
  • 35 Ny T, Sawdey M, Lawrence D, Millan JL, Loskutoff DJ. Cloning and sequence of a cDNA coding for the human β-migrating endothelial cell-type plasminogen activator inhibitor. Proc Natl Acad Sci USA 1986; 83: 6776-6780
    CrossrefPubMedGoogle Scholar
  • 36 Davis III AE, Whitehead AS, Harrison RA, Dauphinais A, Bruns GA P, Cicardi M, Rosen FS. Human inhibitor of the first component of complement, C1: Characterization of cDNA clones and localization of the gene to chromosome 11. Proc Natl Acad Sci USA 1986; 83: 3161-3165
    CrossrefPubMedGoogle Scholar
  • 37 Flink IL, Bailey TJ, Gustafson TA, Markham BE, Morkin E. Complete amino acid sequence of human thyroxine-binding globulin deduced from cloned DNA: Close homology to the serine antiproteases. Proc Natl Acad Sci USA 1986; 83: 7708-7712
    CrossrefPubMedGoogle Scholar
  • 38 Kageyama R, Ohkubo H, Nakanishi S. Primary structure of human preangiotensinogen deduced from the cloned cDNA sequence. Biochemistry 1984; 23: 3603-3609
    CrossrefPubMedGoogle Scholar
  • 39 Kurachi K, Chandra T, Friezner-Degen SJ, White TT, Marchioro TL, Woo SL C, Davie EW. Cloning and sequence of cDNA coding for α1-antitrypsin. Proc Natl Acad Sci USA 1981; 78: 6826-6830
    CrossrefPubMedGoogle Scholar
  • 40 Ohkubo H, Kageyama R, Ujihara M, Hirose T, Inayama S, Nakanishi S. Cloning and sequence analysis of cDNA for rat angiotensinogen. Proc Natl Acad Sci USA 1983; 80: 2196-2200
    CrossrefPubMedGoogle Scholar
  • 41 McReynolds L, O’Malley BW, Nisbett AD, Fothergill JE, Givol D, Fields S, Robertson M, Brownlee GG. Sequence of chicken ovalbumin mRNA. Nature 1978; 273: 723-728
    CrossrefPubMedGoogle Scholar
  • 42 Owen MC, Brennan SO, Lewis JH, Carrell RW. Mutation of antitrypsin to antithrombin α1-antitrypsin Pittsburgh (358 Met → Arg), a fatal bleeding disorder. N Engl J Med 1983; 309: 694-698
    CrossrefPubMedGoogle Scholar
  • 43 Fitch W, Margoliash E. Construction of phylogenetic trees. Science 1967; 155: 279-284
    CrossrefPubMedGoogle Scholar
  • 44 Prochownik EV, Bock SC, Orkin SH. Intron structure of the human antithrombin III gene differs from that of other members of the serine protease inhibitor superfamily. J Biol Chem 1985; 260: 9609-9612
    PubMedGoogle Scholar
  • 45 Loebermann H, Tokuoka R, Deisenhofer J, Huber R. Human α1-proteinase inhibitor: crystal structure analysis of two crystal modifications, molecular model and preliminary analysis of the implications for function. J Mol Biol 1984; 177: 531-556
    CrossrefPubMedGoogle Scholar
  • 46 Toma K, Yamamoto S, Deyashiki Y, Suzuki K. Three-dimensional structure of protein C inhibitor predicted from structure of α1-antitrypsin with computer graphics. Protein Eng 1987; 1: 471-475
    CrossrefPubMedGoogle Scholar
  • 47 Toma K. Simple protein model building tool. J Mol Graphics 1987; 5: 101-102
    CrossrefPubMedGoogle Scholar
  • 48 Marquart M, Walter J, Deisenhofer J, Bode W, Huber R. The geometry of the reactive site and of the peptide groups in trypsin, trypsinogen and its complexes with inhibitors. Acta Cryst 1983; B 39: 480-490
    CrossrefPubMedGoogle Scholar
  • 49 Deyashiki N, Suzuki K. Expression of cDNA coding for the human protein C inhibitor. J Jpn Biochem Soc 1987; 59: 821 (abstr)
    PubMedGoogle Scholar
  • 50 Peterson CB, Blackburn MN. Isolation and characterization of an antithrombin III variant with reduced carbohydrate content and enhanced heparin binding. J Biol Chem 1985; 260: 610-615
    PubMedGoogle Scholar
  • 51 Hill RE, Shaw RK, Boyd PA, Baumann H, Hastie ND. Plasma protease inhibitors in mouse and human: divergence within the reactive centre regions. Nature 1984; 311: 175-177
    CrossrefPubMedGoogle Scholar
  • 52 Hejgaard J, Rasmussen SK, Brandt A, Svendson J. Sequence homology between barley endosperm protein and protease inhibitors of the α-antitrypsin family. FEBS Lett 1985; 180: 89-94
    CrossrefPubMedGoogle Scholar