Hoyer L W, Rizza C R, Tuddenham E G, Carta C A, Armitage H, Rotblat F
Br J Haematol. 1983 Nov;55(3):493-507. doi: 10.1111/j.1365-2141.1983.tb02165.x.
The von Willebrand factor antigen (factor VIII-related antigen, VIIIR:Ag) multimer pattern has been analysed by SDS-agarose electrophoresis of plasmas from 116 patients (47 families) with von Willebrand's disease. In addition to previously recognized patterns, a subclassification was established between plasmas that had a type Ia pattern (VIIIR:Ag multimer pattern like that of normal plasma) and those that had a type Ib pattern in which there was a relative reduction in the concentration of the larger VIIIR:Ag multimers even though all multimeric forms were present. The different patterns were consistent within families and were inherited by autosomal dominant transmission. Von Willebrand's disease heterogeneity was apparent in the distribution of these plasmas: type Ia, 43 patients in 18 families; type Ib, 39 patients in 15 families; type II, 22 patients in 10 families, one of which was further classified as type IIB, one of which was type IIC, and three were IIA. Seven patients with severe von Willebrand's disease were also studied. In general, the interpretation of SDS-agarose multimer patterns corresponded to those previously obtained by crossed immunoelectrophoresis, but the former technique was more sensitive and could identify differences that were not apparent by crossed immunoelectrophoresis.
通过对116例（47个家系）血管性血友病患者血浆进行SDS-琼脂糖电泳，分析了血管性血友病因子抗原（VIII因子相关抗原，VIIIR:Ag）的多聚体模式。除了先前公认的模式外，还在具有Ia型模式（VIIIR:Ag多聚体模式与正常血浆相似）的血浆和具有Ib型模式的血浆之间建立了亚分类，在Ib型模式中，即使所有多聚体形式都存在，但较大的VIIIR:Ag多聚体浓度相对降低。不同模式在家族内是一致的，并通过常染色体显性遗传传递。血管性血友病的异质性在这些血浆的分布中很明显：Ia型，18个家系中的43例患者；Ib型，15个家系中的39例患者；II型，10个家系中的22例患者，其中1例进一步分类为IIB型，1例为IIC型，3例为IIA型。还研究了7例严重血管性血友病患者。一般来说，SDS-琼脂糖多聚体模式的解释与先前通过交叉免疫电泳获得的结果一致，但前一种技术更敏感，能够识别交叉免疫电泳不明显的差异。
