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Plasminogen San Antonio: an abnormal plasminogen with a more cathodic migration, decreased activation and associated
1Department of Pathology, Beth Israel Hospital, Boston, MA 02215.
Abstract:
An abnormal plasminogen (San Antonio) has been isolated from a patient with axillary vein thrombosis. A decreased level of fibrinolytic activity was detected in both plasma and a purified system. The molecular abnormalities were investigated with both functional and immunological tests. Slightly decreased antigen concentration was noted in plasma. By crossed immunoelectrophoresis, the patient and his two children had a second small arc and the primary arc migrated more cathodically. A distinct isozyme was detected in the abnormal plasminogen. Functionally, this abnormal plasminogen is characterized by failure to enhance maximal conversion to plasmin, especially by plasminogen activators, which are enhanced by fibrin or fibrin degradation products. The proband and his children are heterozygous for this abnormal plasminogen.
Insights
An abnormal plasminogen variant, linked to axillary vein thrombosis, shows reduced fibrinolytic activity. This genetic defect impairs plasminogen conversion, affecting blood clot breakdown.
Area of Science:
- Biochemistry
- Hematology
- Molecular Biology
Background:
- Fibrinolytic system is crucial for dissolving blood clots.
- Plasminogen is a key protein in the fibrinolytic cascade.
- Thrombosis can result from dysfunctions in the fibrinolytic system.
Purpose of the Study:
- To investigate the molecular basis of an abnormal plasminogen (San Antonio) found in a patient with axillary vein thrombosis.
- To characterize the functional and immunological properties of this abnormal plasminogen.
- To determine the inheritance pattern of the abnormal plasminogen.
Main Methods:
- Isolation and purification of abnormal plasminogen.
- Functional assays to assess plasminogen activation.
- Immunological techniques including crossed immunoelectrophoresis.
- Analysis of plasma fibrinolytic activity.
Main Results:
- A novel abnormal plasminogen (San Antonio) was identified in a patient with axillary vein thrombosis.
- Reduced fibrinolytic activity was observed in plasma and purified systems.
- Crossed immunoelectrophoresis revealed a distinct isozyme and cathodic shift in the primary arc.
- The abnormal plasminogen exhibited impaired enhancement of plasmin conversion by activators.
Conclusions:
- The abnormal plasminogen (San Antonio) is associated with decreased fibrinolytic activity and an increased risk of thrombosis.
- The molecular defect affects plasminogen activation, particularly in the presence of fibrin or its degradation products.
- The proband and his children are heterozygous carriers, indicating an autosomal inheritance pattern.