Journal
THROMBOSIS RESEARCH
Volume 119, Issue 1, Pages 1-13Publisher
PERGAMON-ELSEVIER SCIENCE LTD
DOI: 10.1016/j.thromres.2005.12.015
Keywords
factor VIII; molecular structure; mutation; bleeding disorders
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Factor VIII (FVIII) is a key component of the fluid phase of the blood coagulation system. The proteases efficiently cleave FVIII at three sites, two within the heavy and one within the light chain resulting in alteration of its covalent structure and conformation and yielding the active cofactor, FVIIIa. FVIIIa is a trimer composed of A(1), A(2) and A(3)-C-1-C-2 subunits. The role of FVIIIa is to markedly increase the catalytic efficiency of factor IXa in the activation of factor X. Variants of these factors frequently also lead to severe bleeding disorders. (c) 2006 Elsevier Ltd. All rights reserved.
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