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Hemostasis following tissue injury involves the deployment of essential plasma procoagulants (prothrombin and Factors X, IX, V and VIII), which are involved in a blood coagulation cascade that leads to the formation of insoluble Fibrin clots and the promotion of platelet aggregation. Coagulation Factor VII (serum prothrombin conversion accelerator, proconvertin, F7, Factor VII) is a 406 amino acid, vitamin K-dependent, single chain serine protease that is synthesized in the liver and circulates as an inactive precursor. Factor IX A, Factor X A, Factor XII A or Thrombin-mediated proteolytic cleavage of Factor VII at Arg 152-Ile 153 generates Factor VII A, an active serine protease composed of a catalytic heavy chain disulfide linked to a light chain, containing two EGF-like domains. Mutations at the F7 locus that lead to Factor VII deficiencies are generally asymptomatic or phenotypically uncharacterized, with hemorrhagic diathesis occurring at extremely low levels.
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