Fang Hong, Wang Lemin, Wang Hongbao
Department of Cardiology, Tongji Hospital, Tongji University, Shanghai 200065, China.
Thromb Res. 2007;119(1):1-13. doi: 10.1016/j.thromres.2005.12.015. Epub 2006 Feb 17.
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 A1, A2 and A3-C1-C2 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.
凝血因子VIII（FVIII）是血液凝固系统液相的关键组成部分。蛋白酶可在三个位点有效切割FVIII，其中两个位于重链内，一个位于轻链内，导致其共价结构和构象发生改变，产生活性辅因子FVIIIa。FVIIIa是一种由A1、A2和A3-C1-C2亚基组成的三聚体。FVIIIa的作用是显著提高因子IXa激活因子X的催化效率。这些因子的变体通常也会导致严重的出血性疾病。
