This gene encodes a member of the serine proteinase inhibitor (serpin) superfamily. This member is the principal inhibitor of tissue plasminogen activator (tPA) and urokinase (uPA), and hence is an inhibitor of fibrinolysis. Defects in this gene are the cause of plasminogen activator inhibitor-1 deficiency (PAI-1 deficiency), and high concentrations of the gene product are associated with thrombophilia. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Sep 2009],disease:Defects in SERPINE1 are the cause of plasminogen activator inhibitor-1 deficiency (PAI-1 deficiency) [MIM:173360]. This deficiency is characterized by abnormal bleeding due to SERPINE1 defect in the plasma.,disease:High concentrations of SERPINE1 have been associated with thrombophilia [MIM:188050], an autosomal dominant disorder in which affected individuals are prone to develop serious spontaneous thrombosis.,function:This inhibitor acts as bait for tissue plasminogen activator, urokinase, and protein C. Its rapid interaction with TPA may function as a major control point in the regulation of fibrinolysis.,online information:Plasminogen activator inhibitor-1 entry,PTM:Inactivated by proteolytic attack of the urokinase-type (u-PA) and the tissue-type (TPA), cleaving the 369-Arg-|-Met-370 bond.,similarity:Belongs to the serpin family.,subunit:Interacts with VTN. Binds LRP1B, binding is followed by internalization and degradation.,tissue specificity:Found in plasma and platelets and in endothelial, hepatoma and fibrosarcoma cells.,
