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Ancestor membership does not transfer phenotype values between groups.","importantHtml":""},"sections":[{"bodyHtml":"<p>Complete PAI-1 deficiency is caused by mutations in the SERPINE1 gene. This gene provides instructions for making a protein called plasminogen activator inhibitor 1 (PAI-1). PAI-1 is involved in normal blood clotting (hemostasis). After an injury, clots protect the body by sealing off damaged blood vessels and preventing further blood loss.</p><p>The PAI-1 protein blocks (inhibits) the action of other proteins called plasminogen activators. These proteins promote the dissolution of clots (fibrinolysis). By inhibiting plasminogen activators, the PAI-1 protein helps ensure that clots remain intact until they are no longer needed to stop bleeding.</p><p>The SERPINE1 gene mutations that cause complete PAI-1 deficiency result in the production of a PAI-1 protein that is nonfunctional or that is unstable and quickly broken down. Absence of functional PAI-1 protein allows plasminogen activators to dissolve blood clots prematurely, resulting in the abnormal bleeding associated with this disorder.</p><p class=\"section-source\"><a href=\"https://medlineplus.gov/genetics/condition/complete-plasminogen-activator-inhibitor-1-deficiency/#causes\">MedlinePlus Genetics: Causes</a></p>","key":"forms","title":"What causes Complete plasminogen activator inhibitor 1 deficiency?"},{"bodyHtml":"<p>Individuals with complete PAI-1 deficiency may experience prolonged nosebleeds, excessive bleeding after medical or dental procedures, easy bruising, and significant bleeding into the joints or soft tissues after even a minor injury. Internal bleeding after an injury, especially bleeding around the brain (intracranial hemorrhage), can be life-threatening. Affected females may have excessive bleeding associated with menstruation (menorrhagia) and abnormal bleeding in pregnancy and childbirth.</p><p>In addition to bleeding problems, some people with complete PAI-1 deficiency develop scar tissue in the heart (cardiac fibrosis), which can lead to heart failure.</p><p class=\"section-source\">Source: <a href=\"https://medlineplus.gov/genetics/condition/complete-plasminogen-activator-inhibitor-1-deficiency\">MedlinePlus Genetics</a></p>","key":"overview","title":"Condition overview"}],"sharedSources":[],"slug":"complete-plasminogen-activator-inhibitor-1-deficiency","sourceDocuments":[{"name":"Complete plasminogen activator inhibitor 1 deficiency","reviewed":"2017-10","sections":{"causes":{"fetchedAt":"2026-10-20T20:20:31.003116+00:00","label":"Causes","pageSha256":"5ece96a2230fb4fc6c10e9575d1607d573b17bc17726d2734109aac740985946","sourceUrl":"https://medlineplus.gov/genetics/condition/complete-plasminogen-activator-inhibitor-1-deficiency/#causes","status":"available"}},"source_release":"2026-10-01","url":"https://medlineplus.gov/genetics/condition/complete-plasminogen-activator-inhibitor-1-deficiency"}],"sourceNote":"MedlinePlus descriptions and Causes; original HPO annotations.","takeawayHtml":"Cause explanations are from MedlinePlus Genetics. 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