The factor VIII protein and its function.

  • Anna Mazurkiewicz-Pisarek Institute of Biotechnology and Antibiotics, Bioengineering Department, Warszawa, Poland.;
  • Grażyna Płucienniczak Institute of Biotechnology and Antibiotics, Bioengineering Department, Warszawa, Poland.;
  • Tomasz Ciach Warsaw University of Technology, Faculty of Chemical and Process Engineering, Department of Biotechnology and Bioprocessor Engineering, Warszawa, Poland.;
  • Andrzej Płucienniczak Institute of Biotechnology and Antibiotics, Bioengineering Department, Warszawa, Poland.;

Abstract

Factor VIII (FVIII), an essential blood coagulation protein, is a key component of the fluid phase blood coagulation system. Human factor VIII is a single chain of about 300 kDa consisting of domains described as A1-A2-B-A3-C1-C2. The protein undergoes processing prior to secretion into blood resulting in a heavy chain of 200 kDa (A1-A2-B) and a light chain of 80 kDa (A3-C1-C2) linked by metal ions. The role of factor VIII is to increase the catalytic efficiency of factor IXa in the activation of factor X. Variants of these factors lead frequently also to severe bleeding disorders.
Published
2016-01-28
Section
Articles