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Plasma Proteins
The research program of the Department of Plasma Proteins focuses on the biology and function of blood coagulation factors that include factor VIII, factor IX, Von Willebrand factor (VWF) and ADAMTS13. Factor VIII is absent in patients with the congenital bleeding disorder hemophilia A whereas factor IX is lacking in patients with hemophilia B. Treatment with highly purified factor VIII and factor IX preparations provide an adequate therapy for hemophilia. Structure-function analysis employing molecular modeling is utilized to increase our understanding of the biochemistry of these coagulation factors with current emphasis on their interactions with clearance receptors that affect their half-life in the circulation. Patients with von Willebrand disease lack functional VWF. Emphasis is on the biosynthesis of VWF in endothelial cell which requires a number of highly specific post-translational modifications that ultimately results in regulated release of polymerized of VWF from intracellular storage pools. Polymer size of VWF is controlled by the recently identified VWF cleaving protease ADAMTS13 which is lacking in patient with thrombotic thrombocytopenic purpura (TTP). Current studies are addressing the characteristics of human antibodies that develop in these patients. Presently, research is organized in four subthemes that address different aspects of blood coagulation:

  • Biosynthesis of the factor VIII-von Willebrand factor complex
  • Structure and function of hemostatic proteins
  • Cellular receptors involved in coagulation factor clearance
  • Immune respons to hemostatic proteins

    A multidisciplinary approach is used within the different lines of research that involves molecular modeling guided protein biochemistry, molecular and cell biology, phage display and culturing of primary endothelial cells derived from umbilical cords.


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