Robbins K C
Department of Medicine, University of Chicago Medical Center/Michael Reese Hospital and Medical Center, Ill.
Enzyme. 1988;40(2-3):70-8. doi: 10.1159/000469148.
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.
这篇关于异常纤溶酶原血症的综述描述了纤溶酶原基因多态性与异常纤溶酶原血症之间日益密切的关系。文中讨论了在美国、日本和欧洲的八个家族中发现的纤溶酶原变体。详细描述了用于鉴定异常纤溶酶原的方法。这些方法包括：（a）纤溶酶原功能与抗原比率；（b）几种纤溶酶原激活剂（如尿激酶、链激酶以及纤溶酶轻链（B）链 - 链激酶复合物）的纤溶酶生成速率；（c）血浆和纯化纤溶酶原的等电形式。对包括纤溶酶形成在内的功能缺陷（包括纤溶酶原激活参数的动力学）进行了综述。描述了在一个家族（枥木I型）中发现的分子缺陷，即发现了一个单氨基酸取代。最后，对异常纤溶酶原变体之间缺乏相关性进行了综述。这些变体分为两类：一类是完全没有起作用的活性中心，另一类是具有不同的纤溶酶原激活参数动力学。
