A serine protease natural anticoagulant that inhibits thrombin and other coagulation factors.
A 58-kDa molecule.
Contains 432 amino acids.
Belongs to the serpin family of inhibitors, which includes heparin cofactor II, alpha2-antiplasmin, plasmin activator inhibitor-1, alpha-antitrypsin, and C1 inhibitor.
Forms a 1:1 complex with target enzyme, which is irreversible and the complex is cleared by the liver.
Two forms an alpha form (90%), glycosylated at all positions, and a beta form (10%) not glycosylated at position Asn.
Interaction between AT and blood vessel walls and glycosaminoglycans.
When bound to heparin it accelerates the inactivation of thrombin (factor IIa) as well as factor IXa, factor Xa, and factor XIa that participate in thrombin generation.
Deficiency inherited in an autosomal dominant fashion with an estimated frequency of 1 in 2000.
Has anti-inflammatory actions.
Available as Thrombate III a highly purified antithrombin concentrate fractionated from human plasma.
Thrombate III mimics the role of endogenous antithrombin.
Thrombate III approved for patients with hereditary antithrombin III deficiency.