Mikami S, Ueda M, Yasui M, Takahashi Y, Nishino M, Fukui H
Thromb Haemost. 1983 Apr 28;49(2):87-90.
The nature of sugar chain of factor VIII/von Willebrand factor in plasma of normal subjects and patients with von Willebrand's disease (vWd) was examined by crossed affinoimmunoelectrophoresis using anti-human factor VIII rabbit serum, with inserted Ricinus communis agglutinin-120 (RCA-120) agarose layer (RCA - CIE). Molecular weights of factor VIII-related antigen (VIIIR:Ag) were estimated by SDS polyacrylamide gel electrophoresis - crossed affinoimmunoelectrophoresis (SDS PAGE - RCA - CIE). VIIIR:Ag, in normal plasma and in classical form of vWd, showed two precipitin peaks on RCA - CIE. The slower moving component of VIIIR:Ag with molecular weights over 3 x 10(6) daltons from normal subjects and patients with classical form of vWd showed a high affinity for RCA-120. The faster moving component of VIIIR:Ag below 3 x 10(6) daltons from the above-mentioned subjects and patients with a variant form (Type IIA) showed a very weak affinity for RCA-120. These results suggested that all of VIIIR:Ag in these variant cases may have a deficiency of galactose residues reactive with RCA, in addition to an incomplete polymerization of VIIIR:Ag, similar to that of the faster moving component of normal subjects.
采用抗人因子VIII兔血清，通过插入蓖麻凝集素-120（RCA-120）琼脂糖层的交叉亲和免疫电泳（RCA - CIE），检测正常人和血管性血友病（vWd）患者血浆中因子VIII/血管性血友病因子的糖链性质。通过SDS聚丙烯酰胺凝胶电泳-交叉亲和免疫电泳（SDS PAGE - RCA - CIE）估计因子VIII相关抗原（VIIIR:Ag）的分子量。VIIIR:Ag在正常血浆和经典型vWd中，在RCA - CIE上显示出两个沉淀素峰。正常人和经典型vWd患者中分子量超过3×10⁶道尔顿的VIIIR:Ag的较慢移动成分对RCA-120具有高亲和力。上述受试者和变异型（IIA型）患者中低于3×10⁶道尔顿的VIIIR:Ag的较快移动成分对RCA-120的亲和力非常弱。这些结果表明，除了VIIIR:Ag聚合不完全外，这些变异病例中的所有VIIIR:Ag可能都缺乏与RCA反应的半乳糖残基，这与正常受试者的较快移动成分类似。
