Enayat M S, Hill F G
J Clin Pathol. 1983 Aug;36(8):915-9. doi: 10.1136/jcp.36.8.915.
A method for multimeric analysis of factor VIII related antigen/von Willebrand protein is described. By modifying an existing method the technique has been simplified and optimised so that the different molecular forms of factor VIII related antigen and their triplet structure can be visualised. Clear differences can be seen between patterns in normals and type IIA von Willebrand's disease variants in that the latter lack high and intermediate multimers and also have a different configuration to the triplet pattern in the lower molecular weight multimers. Differences in triplet pattern can also be seen in different type IIA patients. The advantages and further application of this method for investigating the molecular defect in von Willebrand's disease are discussed.
本文描述了一种用于因子VIII相关抗原/血管性血友病因子多聚体分析的方法。通过改进现有方法,该技术得以简化和优化,从而能够观察到因子VIII相关抗原的不同分子形式及其三联体结构。正常人和IIA型血管性血友病变体的模式之间存在明显差异,后者缺乏高分子量和中等分子量的多聚体,并且在低分子量多聚体中的三联体模式也具有不同的构型。在不同的IIA型患者中也可以看到三联体模式的差异。本文还讨论了该方法在研究血管性血友病分子缺陷方面的优势和进一步应用。