The factor VIII protein and its function.
Author(s) -
Anna Mazurkiewicz-Pisarek,
Grażyna Płucienniczak,
Tomasz Ciach,
Andrzej Płucienniczak
Publication year - 2016
Publication title -
acta biochimica polonica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.452
H-Index - 78
eISSN - 1734-154X
pISSN - 0001-527X
DOI - 10.18388/abp.2015_1056
Subject(s) - factor ixa , coagulation , immunoglobulin light chain , chemistry , coagulation system , secretion , tissue factor , biochemistry , factor x , microbiology and biotechnology , medicine , immunology , biology , platelet , antibody , thrombin
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.
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