Dysfibrinogenemia (Fibrinogen Dusard) Associated with Impaired Fibrin-Enhanced Plasminogen Activation

H R Lijnen; J Soria; C Soria; D Collen; J P Caen

doi:10.1055/s-0038-1661033

Thrombosis and Haemostasis, Inhaltsverzeichnis

Thromb Haemost 1984; 51(01): 108-109
DOI: 10.1055/s-0038-1661033

Original Article

Schattauer GmbH Stuttgart

Dysfibrinogenemia (Fibrinogen Dusard) Associated with Impaired Fibrin-Enhanced Plasminogen Activation

H R Lijnen

The Center for Thrombosis and Vascular Research, Department of Medical Research, University of Leuven, Leuven, Belgium

,

J Soria

^*The Unité de Recherches de Thrombose Expérimentale, INSERM U.150, LA 334, CNRS, Hôpital Lariboisière, Paris, France

,

C Soria

^*The Unité de Recherches de Thrombose Expérimentale, INSERM U.150, LA 334, CNRS, Hôpital Lariboisière, Paris, France

,

D Collen

The Center for Thrombosis and Vascular Research, Department of Medical Research, University of Leuven, Leuven, Belgium

,

J P Caen

^*The Unité de Recherches de Thrombose Expérimentale, INSERM U.150, LA 334, CNRS, Hôpital Lariboisière, Paris, France

› Institutsangaben

Abstract

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Summary

The fibrin-mediated enhancement of the activation of plasminogen by tissue-type plasminogen activator observed with normal fibrin, is strongly decreased with fibrin Dusard, although the binding of tissue-type plasminogen activator to this fibrin is normal. This impaired fibrin-mediated plasminogen activation is most likely related to the history of recurrent thrombosis and pulmonary embolism observed in this family.

Key Words

Dysfibrinogenemia - Impaired fibrinolysis - Fibrinogen Dusard

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Referenzen

References
1 Wiman B, Collen D. Molecular mechanism of physiological fibrinolysis. Nature 1978; 272: 549-550
2 Collen D. On the regulation and control of fibrinolysis. Thromb Haemostas 1980; 43: 77-89
3 Hoylaerts M, Rijken DC, Lijnen HR, Collen D. Kinetics of the activation of plasminogen by human tissue plasminogen activator. Role of fibrin J Biol Chem 1982; 257: 2912-2919
4 Soria J, Soria C, Caen JP. A new type of congenital dysfib- rinogenaemia with defective fibrin lysis. Dusard syndrome: possible relation to thrombosis Br J Haematol 1983; 53: 575-586
5 Blombäck B, Blombäck M. Purification of human and bovine fibrinogen. Ark Kemi 1956; 10: 415-443
6 Deutsch DG, Mertz ET. Plasminogen: purification from human plasma by affinity chromatography. Science 1970; 170: 1095-1096
7 Wallén P, Wiman B. Characterization. I. On the relationship between different molecular forms of plasminogen demonstrated in plasma and found in purified preparations. Biochim Biophys Acta 1970; 221: 20-30
8 Rijken DC, Collen D. Purification and characterization of the plasminogen activator secreted by human melanoma cells in culture. J Biol Chem 1981; 256: 7035-7041
9 Astrup T, Müllertz S. The fibrin plate method for estimating fibrinolytic activity. Arch Biochem Biophys 1952; 40: 346-351
10 Rånby M, Wallén P. A sensitive parabolic rate assay for the tissue plasminogen activator. In: Progress in Fibrinolysis. Davidson JF, Nilsson IM, Asted B. (eds) Churchill Livingstone; Edinburg: 1981. V 233-235
11 Lijnen HR, Collen D. Chromogenic substrate assays of components of the fibrinolytic system. Acta Chir Scand 1982; suppl (Suppl. 509) 129-132
12 Camiolo SM, Thorsen S, Astrup T. Fibrinogenolysis and fibrinolysis with tissue plasminogen activator, urokinase, streptokinase-activated human globulin, and plasmin. Proc Soc Exp Biol Med 1971; 138: 277-280
13 Wallén P. Activation of plasminogen with urokinase and tissue activator. In: Thrombosis and Urokinase. Paoletti R, Sherry S. (eds) Academic Press; London: 1977. pp 91-102