SERPIND1

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SERPIND1

A gene on chromosome 22q11.21 that encodes a member of the serine protease inhibitor (serpin) family that rapidly inhibits thrombin in the presence of dermatan sulfate or heparin. SERPIND1 shares homology with antithrombin III and other members of the alpha 1-antitrypsin superfamily.

Molecular pathology
SERPIND1 mutations are associated with heparin cofactor-II deficiency.