Chediak J, Peake I, Bloom A
Thromb Haemost. 1983 Aug 30;50(2):509-12.
Gel filtration (sepharose 2B CL) patterns of factor VIII coagulant antigen (VIIIC:Ag) and factor VIII related antigen (VIIIR:Ag) were obtained using normal plasma and plasma from patients with von Willebrand's disease. The latter group consisted of five individuals with normal mobility of factor VIIIR:Ag on cross-immunoelectrophoresis (Type I) and five others with abnormal (increased) mobility (Type II). Results showed that the elution of VIIIC:Ag was delayed in those subjects whose ratio of VIIIR:Ag to VIIIC:Ag was reduced. It has previously been reported that VIIIR:Ag exerts a stabilizing influence on the coagulant activity of factor VIII (VIII:C); our data suggests that when VIIIR:Ag is deficient, abnormal (low molecular weight) forms of VIIIC:Ag circulate.
利用正常血浆和血管性血友病患者的血浆,获得了凝血因子VIII促凝抗原(VIIIC:Ag)和因子VIII相关抗原(VIIIR:Ag)的凝胶过滤(琼脂糖2B CL)图谱。后一组包括5名在交叉免疫电泳中因子VIIIR:Ag迁移率正常的个体(I型)和另外5名迁移率异常(增加)的个体(II型)。结果显示,在那些VIIIR:Ag与VIIIC:Ag比值降低的受试者中,VIIIC:Ag的洗脱延迟。此前已有报道称,VIIIR:Ag对因子VIII(VIII:C)的凝血活性具有稳定作用;我们的数据表明,当VIIIR:Ag缺乏时,异常(低分子量)形式的VIIIC:Ag会在循环中出现。