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.,
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