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Thromb Haemost 1986; 55(03): 396-401
DOI: 10.1055/s-0038-1661572
Original Article
Schattauer GmbH Stuttgart

Investigation of a Congenital Abnormal Plasminogen, Frankfurt I, and Its Relationship to Thrombosis

I M Scharrer
1   The Department of Internal Medicine, University Flospital1, Frankfurt/M, FRG
,
R C Wohl
2   The Division of Experimental Pathology, Department of Pathology, Michael Reese Hospital and Medical Center, Chicago, IL, USA
2   The Departments of Pathology and Medicine, Pritzker School of Medicine, University of Chicago, Chicago, USA
,
V Hach
1   The Department of Internal Medicine, University Flospital1, Frankfurt/M, FRG
,
L Sinio
2   The Division of Experimental Pathology, Department of Pathology, Michael Reese Hospital and Medical Center, Chicago, IL, USA
2   The Departments of Pathology and Medicine, Pritzker School of Medicine, University of Chicago, Chicago, USA
,
I Boreisha
2   The Division of Experimental Pathology, Department of Pathology, Michael Reese Hospital and Medical Center, Chicago, IL, USA
2   The Departments of Pathology and Medicine, Pritzker School of Medicine, University of Chicago, Chicago, USA
,
K C Robbins
2   The Division of Experimental Pathology, Department of Pathology, Michael Reese Hospital and Medical Center, Chicago, IL, USA
2   The Departments of Pathology and Medicine, Pritzker School of Medicine, University of Chicago, Chicago, USA
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Publikationsverlauf

Received 16. Juli 1985

Accepted 21. April 1986

Publikationsdatum:
18. Juli 2018 (online)

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Summary

A new abnormal plasminogen, Frankfurt I, has been identified in the plasma of a 42 year-old male patient who has recurrent deep vein thrombosis. Clinical laboratory data showed normal hemostasis test results. Since plasma plasmin generation rates gave low values, the fibrinolytic system was analyzed for a possible fibrinolytic system defect. Functional and antigen plasminogen concentrations both in the plasma and with the isolated, purified plasminogen showed that only 49% of the antigen concentration had potential functional active sites. Also, a reduced antigen concentration was found in both the propositus, and his mother (46% active sites).

Sodium dodecyl sulfate polyacrylamide gel electrophoresis of the purified Frankfurt I plasminogen showed a normal native Glu-plasminogen band. Crossed-immunoelectrophoresis revealed a peak with normal size and shape, but displaced with respect to normal Glu-plasminogen toward the anode, i. e., was, as a whole, more negatively charged. Isoelectric focusing followed by zymog-raphy on a agarose-fibrin plate proved this observation, but did not indicate a separation of the normal from the abnormal plasminogen molecular species, also, fewer bands were found in the abnormal plasminogen isozyme pattern.

Kinetic studies of Frankfurt I Glu-plasminogen and plasmin led to the conclusion that most of the functional abnormality is related to absence of active sites in half of the molecules. The plasmin generated was very unstable in the absence of stabilizing ligands and/or substrates. After reduction, the plasmin was completely converted to the typical two plasmin chains, A and B.

Keywords

Abnormal plasminogens - Variant plasminogen Frankfurt I - Deep vein thrombosis - Fibrinolysis
 

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