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Full-Text Articles in Life Sciences
The Tertiary Structure And Domain Organization Of Coagulation Factor Viii, P. Clint Spiegel, Betty W. Shen, Chong-Hwan Chang, Jae-Wook Huh, Jeanman Kim, Young-Ho Kim, Barry L. Stoddard
The Tertiary Structure And Domain Organization Of Coagulation Factor Viii, P. Clint Spiegel, Betty W. Shen, Chong-Hwan Chang, Jae-Wook Huh, Jeanman Kim, Young-Ho Kim, Barry L. Stoddard
Chemistry Faculty and Staff Publications
Factor VIII (fVIII) is a serum protein in the coagulation cascade that nucleates the assembly of a membrane-bound protease complex on the surface of activated platelets at the site of a vascular injury. Hemophilia A is caused by a variety of mutations in the factor VIII gene and typically requires replacement therapy with purified protein. We have determined the structure of a fully active, recombinant form of factor VIII (r-fVIII), which consists of a heterodimer of peptides, respectively containing the A1-A2 and A3-C1-C2 do- mains. The structure permits unambiguous modeling of the relative orientations of the 5 domains of r-fVIII. …