Thrombin-activable Factor X Re-establishes an Intrinsic Amplification in Tenase-deficient Plasmas

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Thrombin-activable factor X re-establishes an intrinsic amplification in tenase-deficient plasmas.

Classical hemophilia results from a defect of the intrinsic tenase complex, the main factor X (FX) activator. Binding of factor VIIa to tissue factor triggers coagulation, but little amplification of thrombin production occurs. Handling of hemophilia by injection of the deficient or missing (thus foreign) factor often causes immunological complications. Several strategies have been designed to ...

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Heparin inhibits the intrinsic tenase complex by interacting with an exosite on factor IXa.

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The heparin-binding exosite is critical to allosteric activation of factor IXa in the intrinsic tenase complex: the role of arginine 165 and factor X.

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ژورنال

عنوان ژورنال: Journal of Biological Chemistry

سال: 2005

ISSN: 0021-9258

DOI: 10.1074/jbc.m507846200