Dysplasminogenemias

This review on dysplasminogenemias describes a growing relationship between genetic polymorphisms of plasminogen and dysplasminogenemias. Plasminogen variants found in eight families in America, Japan and Europe are discussed. Methods used to identify abnormal plasminogens are described in detail. These methods include (a) plasminogen functional to antigen ratios, (b) plasmin generation rates with several plasminogen activators, e.g. urokinase, streptokinase, and the plasmin light (B) chain.streptokinase complex, and (c) plasma and purified plasminogen isoelectric forms. The functional defect including plasminogen kinetics of activation parameters are reviewed, including the formation of plasmin. The molecular defect found in one family, Tochigi I, is described, a single amino acid substitution was found. Finally, the lack of relationships between the abnormal plasminogen variants is reviewed. The variants fall into two classes: one class with a complete absence of a functioning active center, and the second class with different plasminogen kinetics of activation parameters.