Thromb Haemost 1979; 41(04): 709-713
DOI: 10.1055/s-0038-1646830
Original Articles
Schattauer GmbH Stuttgart

Fibrinogen Zurich I: Impaired Release of Fibrinopeptide A

V Hofmann
The Thrombosis Laboratory, Department of Medicine, Inselspital, University of Berne, 3010 Berne and the Coagulation Laboratory, Department of Medicine, University of Zurich, Switzerland
,
W P Gati
The Thrombosis Laboratory, Department of Medicine, Inselspital, University of Berne, 3010 Berne and the Coagulation Laboratory, Department of Medicine, University of Zurich, Switzerland
,
P W Straub
The Thrombosis Laboratory, Department of Medicine, Inselspital, University of Berne, 3010 Berne and the Coagulation Laboratory, Department of Medicine, University of Zurich, Switzerland
› Author Affiliations
Further Information

Publication History

Publication Date:
09 July 2018 (online)

Summary

Fibrinogen Zurich I is characterized by an abnormal fibrin monomer polymerization. It consists of two fractions of molecules, one with a normal aggregation and one not aggregating at all and interfering with the aggregation of the normal population. Using a radioimmunoassay for fibrinopeptide A, only approximately half of the expected fibrinopeptide A could be recovered after thrombin or Defibrase proteolysis. The defective fibrinopeptide A release could be confirmed by measurement of the N-terminal Gly/Tyr ratio. It is likely that the abnormal fibrin monomer aggregation of the abnormal fraction of fibrinogen Zurich I is due to the defective fibrinopeptide A release of this fraction.

 
  • References

  • 1 Al-Mondhiry HA B, Bilezikian SB, Nossel HL. 1975; Fibrinogen New York -an abnormal fibrinogen associated with thromboembolism:Functional evaluation. Blood 45: 607
  • 2 Beck EA, Shainoff JR, Vogel A, Jackson DP. 1971; Functional evaluation of an inherited abnormal fibrinogen: Fibrinogen “Baltimore”. Journal of Clinical Investigation 50: 1874
  • 3 Blomback B, Vestermark A. 1958; Isolation of fibrinopeptides by chromatography. Arkiv Kemi 12: 173
  • 4 Blombäck B. 1958; Studies on the action of thrombin enzymes on bovine fibrinogen as measured by N-terminal analysis. Arkiv Kemi 72: 321
  • 5 Blombäck M. 1958; Purification of antihemophilic globulin.I. Some studies on the stability of the antihemophilic globulin activity in fraction I-O and a method for its partial separation from fibrinogen. Arkiv Kemi 12: 387
  • 6 Crum ED, Shainoff JR, Graham RC, Ratnoff OD. 1974; Fibrinogen Cleveland II: An abnormal fibrinogen with defective release of fibrinopeptide A. Journal of Clinical Investigation 53: 1308
  • 7 Forman WB, Ratnoff OD, Boyer MH. 1968; An inherited qualitative abnormality in plasma fibrinogen: Fibrinogen ”Cleveland”. Journal of Laboratory and Clinical Medicine 72: 455
  • 8 Funk C, Gmuer J, Herold R, Straub PW. 1971; Reptilase - A new reagent in blood coagulation. British Journal of Haematology 21: 43
  • 9 Gralnick HR, Givelber HM, Shainoff JR, Finlayson JS. 1971; Fibrinogen ”Bethesda”: a congenital dysfibrinogenemia with delayed fibrinopeptide release. Journal of Clinical Investigation 50: 1819
  • 10 Herzig RH, Ratnoff OD, Shainoff JR. 1970; Studies on a procoagulant fraction of southern copperhead snake venom: The preferential release of fibrinopeptide B. Journal of Laboratory and Clinical Medicine 76: 451
  • 11 Hofmann V, Straub PW. 1977; A radioimmunoassay technique for the rapid measurement of human fibrinopeptide A. Thrombosis Research 11: 171
  • 12 Imperato DC, Dettori AG. 1958; Ipofibrinogenemia congenita con fibrinoastenia. Helvetica Paediatrica Acta 13: 380
  • 13 Krause WH, Heene DL, Lasch HG. 1973; Congenital dysfibrinogenemia (Fibrinogen GieBen). Thrombosis et Diathesis Haemorrhagica 29: 547
  • 14 Menache D. 1973; Abnormal fibrinogens. A review. Thrombosis et Diathesis Haemorrhagica 29: 525
  • 15 Soria J, Soria C, Samama M, Poirot E, Kling C. 1972; Fibrinogen ”Troyes”, Fibrinogen ”Metz”. Two new cases of congenital dysfibrinogenemia. Thrombosis et Diathesis Haemorhagica 27: 619
  • 16 Von Felten A, Duckert F, Frick PG. 1966; Familial disturbance of fibrin monomer aggregation. British Journal of Haematology 12: 667
  • 17 Von Felten A, Frick PG, Straub PW. 1969; Studies on fibrin monomer aggregation in congenital dysfibrinogenaemia (Fibrinogen ”Zurich”): Separation of a pathological from a normal fibrin fraction. British Journal of Haematology 16: 353