The factor VIII protein and its function

被引：57

作者：

Mazurkiewicz-Pisarek, Anna ^{[1
]}

Plucienniczak, Grazyna ^{[1
]}

Ciach, Tomasz ^{[2
]}

Plucienniczak, Andrzej ^{[1
]}

机构：

[1] Inst Biotechnol & Antibiot, Bioengn Dept, Warsaw, Poland

[2] Warsaw Univ Technol, Fac Chem & Proc Engn, Dept Biotechnol & Bioprocessor Engn, Warsaw, Poland

来源：

ACTA BIOCHIMICA POLONICA | 2016年 / 63卷 / 01期

关键词：

Factor VIII (FVIII); molecular structure; function; hemophilia A; bleeding disorders; RECOMBINANT FACTOR-VIII; PREVIOUSLY UNTREATED PATIENTS; COAGULATION-FACTOR-VIII; SEVERE HEMOPHILIA-A; B-DOMAIN; FACTOR-IX; CRYSTAL-STRUCTURE; THROMBIN; GENE; INHIBITORS;

D O I：

10.18388/abp.2015_1056

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

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.

引用

页码：11 / 16

页数：6

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