Salem H H, Howard M A, Koutts J, Firkin B G
Br J Haematol. 1985 Mar;59(3):485-96. doi: 10.1111/j.1365-2141.1985.tb07335.x.
A coagulation accelerating factor was purified from the plasma of two patients with glomerulonephritis (GN) who suffered from thrombotic complications. The factor co-purified with factor VIII/von Willebrand factor complex (FVIII/vWf) and under dissociating conditions remained associated with the factor VIII coagulant activity (FVIII). Control purified FVIII/vWf showed no coagulation accelerating activity under the experimental conditions used. The levels of coagulation accelerating factor, FVIII and von Willebrand factor (vWf) were reduced by incubation with rabbit anti-human FVIII/vWf or human anti-FVIII serum indicating a close association of these three activities. Multimeric analysis of the plasma FVIII/vWf complex from the two patients demonstrated a reduction in the high molecular weight multimers and the presence of an additional band not present on analysis of normal FVIII/vWf. It is suggested that the coagulation accelerating factor represents an active form of FVIII which has different in vitro properties to thrombin activated FVIII.
从两名患有血栓形成并发症的肾小球肾炎(GN)患者的血浆中纯化出一种促凝因子。该因子与因子VIII/血管性血友病因子复合物(FVIII/vWf)共同纯化,并且在解离条件下仍与因子VIII凝血活性(FVIII)相关联。在所用的实验条件下,对照纯化的FVIII/vWf未显示促凝活性。用兔抗人FVIII/vWf或人抗FVIII血清孵育后,促凝因子、FVIII和血管性血友病因子(vWf)的水平降低,表明这三种活性密切相关。对两名患者的血浆FVIII/vWf复合物进行多聚体分析,结果显示高分子量多聚体减少,并且在正常FVIII/vWf分析中不存在的额外条带出现。提示促凝因子代表FVIII的一种活性形式,其体外特性与凝血酶激活的FVIII不同。