Download PDF
Thromb Haemost 1993; 70(06): 0894-0899
DOI: 10.1055/s-0038-1649695
Original Article
Clinical Studies
Schattauer GmbH Stuttgart

Hereditary Increase of Plasma Histidine-Rich Glycoprotein Associated with Abnormal Heparin Binding (HRG Eindhoven)

J J M L Hoffmann
1   The Haemostasis Division, Department of Clinical Laboratories, Catharina Hospital, Eindhoven, The Netherlands
,
B C Hennis
2   Gaubius Laboratory IVVO-TNO, Leiden, The Netherlands
,
C Kluft
2   Gaubius Laboratory IVVO-TNO, Leiden, The Netherlands
,
M Vijgen
1   The Haemostasis Division, Department of Clinical Laboratories, Catharina Hospital, Eindhoven, The Netherlands
› Author Affiliations
Further Information

Publication History

Received 26 February 1993

Accepted after revision 19 August 1993

Publication Date:
06 July 2018 (online)

  PDF Download  Permissions and Reprints

Summary

Plasma histidine-rich glycoprotein (HRG) was found to be persistently increased in a patient with a history of recurrent arterial thromboembolic events. The mean concentration was 270% of normal pooled plasma. Increased HRG was found in eight of the 17 relatives studied, but none of them has experienced thrombo-embolism yet. Apparently, increased HRG was hereditary with autosomal dominant inheritance. A significant correlation was found between the increased plasma concentration of the protein and the age of the subjects (P<0.02), whereas no such relation is present in a normal population.

The plasma HRG of the proposita and 9 of her family members displayed abnormal binding to heparin, as assessed in a crossed affinity immuno-electrophoresis system: the usual increase in mobility after binding to heparin was absent. The binding of this variant HRG to plasminogen was normal. This case represents the first abnormal HRG variant reported and it is proposed to designate it: HRG Eindhoven.

 

  • References

  • 1 Heimburger N, Haupt H, Krantz T, Baudner S. Humanserum-proteine mit hoher Affinitét zu Carboxymethylcellulose II. Phy-sikalisch-chemische und immunologische Charakterisierung eines histidinreichen 3,8S-α2-Glykoproteins (CM-Protein I). Hoppe-Seyl Z Phys Chem 1972; 353: 1133-1140
    PubMedGoogle Scholar
  • 2 Koide T, Odani S, Ono T. The N-terminal sequence of human plasma histidine-rich glycoprotein homologous to antithrombin with high affinity for heparin. FEBS Lett 1982; 141: 222-224
    CrossrefPubMedGoogle Scholar
  • 3 Koide T, Foster D, Yoshitake S, Davie EW. Amino acid sequence of human histidine-rich glycoprotein derived from the nucleotide sequence of its cDNA. Biochemistry 1986; 25: 2220-2225
    CrossrefPubMedGoogle Scholar
  • 4 Lijnen HR, Hoylaerts M, Collen D. Heparin binding properties of human histidine-rich glycoprotein. Mechanism and role in the neutralization of heparin in plasma. J Biol Chem 1983; 258: 3803-3808
    PubMedGoogle Scholar
  • 5 Lijnen HR, van HoefB, Collen D. Histidine-rich glycoprotein modulates the anticoagulant activity of heparin in human plasma. Thromb Haemost 1984; 51: 266-268
    Article in Thieme ConnectPubMedGoogle Scholar
  • 6 Lijnen HR, Hoylaerts M, Collen D. Isolation and characterization of a human plasma protein with affinity for the lysine binding sites in plasminogen. Role in the regulation of fibrinolysis and identification as histidine-rich glycoprotei. J Biol Chem 1980; 255: 10214-10222
    PubMedGoogle Scholar
  • 7 Ichinose A, Mimuro J, Koide T, Aoki N. Histidine-rich glycoprotein and α2-plasmin inhibitor in inhibition of plasminogen binding to fibrin. Thromb Res 1984; 33: 401-407
    CrossrefPubMedGoogle Scholar
  • 8 Leung LLK. Interaction of histidine-rich glycoprotein with fibrinogen and fibrin. J Clin Invest 1986; 77: 1305-1311
    CrossrefPubMedGoogle Scholar
  • 9 Lerch PG, Nydegger UE, Kuyas C, Haeberli A. Histidine-rich glycoprotein binding to activated human platelets. Brit J Haematol 1988; 70: 219-224
    CrossrefPubMedGoogle Scholar
  • 10 Castel M, Horellou MH, Conard J, Samama M. Immunochemical determination of histidine-rich glycoprotein in healthy subjects and in a clinical population. In: Progress in Fibrinolysis. Davidson JF, Bachmann F, Bouvier CA, Kruithof EKO. eds. Edinburgh: Chur-chill-Livingstone; 1983: 370-377
    Google Scholar
  • 11 Samama M, Conard J, Castel-Gatey M, Horellou MH. Histidine-rich glycoprotein and deep venous thrombosis. In: Clinical aspects of fibrinolysis and thrombolysis. Jespersen J, Kluft C, Korsgaard O. eds. Esbjerg: South Jutland University Press; 1983: 163-173
    Google Scholar
  • 12 Engesser L, Kluft C, Briët E, Brommer EJP. Familial elevation of plasma histidine-rich glycoprotein in a family with thrombophilia. Brit J Haematol 1987; 67: 355-358
    CrossrefPubMedGoogle Scholar
  • 13 Falkon L, Barí M, Montserrat I, Borell M, Fontcuberta J. Familial elevation of plasma histidine-rich glycoprotein: a case associated with recurrent venous thrombosis and high PAI1 levels. Thromb Res 1992; 66: 265-270
    CrossrefPubMedGoogle Scholar
  • 14 Angles-Cano E, Gris JC, Loyau S, Schved JF. Familial association of high levels of histidine-rich glycoprotein and plasminogen activator inhibitor-1 with venous thromboembolism. J Clin Lab Med 1993; 121: 646-653
    PubMedGoogle Scholar
  • 15 Engesser L, Kluft C, Juhan-Vague I, Briët E, Brommer EJP. Plasma histidine-rich glycoprotein and thrombophilia. Fibrinolysis 1988; 2 (Suppl. 02) 43-46
    PubMedGoogle Scholar
  • 16 Shigekiyo T, Tomonari A, Uno Y, Azuma H, Shunto R, Saito S, Kaneko H, Satoh K, Ueda S. Familial elevation of plasma histidine-rich glycoprotein in a Japanese family. Jap J Thromb Hemost 1991; 2: 530-536
    PubMedGoogle Scholar
  • 17 Leung LLK, Harpel PC, Nachman RL, Rabellino EM. Histidine-rich glycoprotein is present in human platelets and is released following thrombin stimulation. Blood 1983; 62: 1016-1021
    PubMedGoogle Scholar
  • 18 Laurell C-B. Quantitative estimation of proteins by electrophoresis in agarose gel containing antibodies. Anal Biochem 1966; 15: 45-52
    CrossrefPubMedGoogle Scholar
  • 19 Bertina RM, van derLinden IK, Engesser L, Muller HP, Brommer EJP. Hereditary heparin cofactor II deficiency and the risk of development of thrombosis. Thromb Haemost 1987; 57: 196-200
    Article in Thieme ConnectPubMedGoogle Scholar
  • 20 Verheijen JH, Mullaart E, Chang GTC, Kluft C, Wijngaards G. A simple, sensitive spectrophotometric assay for extrinsic (tissue-type) plasminogen activator applicable to measurement in plasma. Thromb Haemost 1982; 48: 266-269
    Article in Thieme ConnectPubMedGoogle Scholar
  • 21 Binnema DJ, van IerselJJL, Dooijewaard G. Quantitation of urokinase antigen in plasma and culture media by use of an ELISA. Thromb Res 1986; 43: 569-577
    CrossrefPubMedGoogle Scholar
  • 22 Kluft C, Los P. Modified crossed immunoelectrophoresis to study with whole plasma the reversible complex formation of histidine-rich glycoprotein with plasminogen. Thromb Haemost 1988; 60: 411-414
    Article in Thieme ConnectPubMedGoogle Scholar
  • 23 Thompson SG, van deLoo J, Haverkate F. Principal results of the EC AT angina pectoris study. Thromb Haemost 1991; 65: 816 (abstract)
    PubMedGoogle Scholar
  • 24 Lijnen HR, Jacobs G, Collen D. Histidine-rich glycoprotein in a normal and a clinical population. Thromb Res 1981; 22: 519-523
    CrossrefPubMedGoogle Scholar
  • 25 Hamsten A, Wiman B, de FaireU, Blombäck M. Increased plasma levels of a rapid inhibitor of tissue plasminogen activator in young survivors of myocardial infarction. N Engl J Med 1985; 313: 1557-1563
    CrossrefPubMedGoogle Scholar
  • 26 Nilsson IM, Ljungnèr H, Tengborn L. Two different mechanisms in patients with venous thrombosis and defective fibrinolysis: low concentration of plasminogen activator or increased concentration of plasminogen activator inhibitor. Brit Med J 1985; 290: 1453-1456
    CrossrefPubMedGoogle Scholar
  • 27 Paramo JA, Colucci M, Collen D, van derWerf F. Plasminogen activator inhibitor in the blood of patients with coronary artery disease. Brit Med J 1985; 291: 573-574
    CrossrefPubMedGoogle Scholar