Complete Deficiency of Plasminogen-Activator Inhibitor Type 1 Due to a Frame-Shift Mutation

Abstract
THE process of plasminogen activation is considered a critical component of diverse biologic systems in humans. This reaction, which is catalyzed in vivo by tissue-type and urokinase-type plasminogen activators, results in the conversion of plasminogen to plasmin. Plasmin has an essential role in maintaining vascular patency by converting fibrin to soluble fibrin-degradation products. In addition, plasminogen activators have been implicated in the regulation of embryogenesis, angiogenesis, ovulation, inflammation, and tumor metastasis,1 suggesting that the plasminogen-activation system is an important mediator of tissue remodeling and cell migration.