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血管性血友病中凝血因子VIII/血管性血友病因子分子大小的异质性

Heterogeneity of molecular size of factor VIII/von Willebrand factor in von Willbrand's disease.

作者信息

Mikami S, Takahashi Y, Nishino M, Okubo Y, Fukui H

出版信息

Thromb Haemost. 1981 Jun 30;45(3):272-5.

PMID:6792740
Abstract

Patterns of VIIIR:AG in the plasma and its fractions, cryoprecipitate and cryosupernatant, from various types of von Willebrand's disease (vWd) were observed by SDS 1.5% polyacrylamide gel electrophoresis - cross immunoelectrophoresis (SDS PAGE - CIE). VIIIR:AG in normal cryoprecipitate showed several precipitin peaks which correspond to molecular weights ranging from 8 X 10(5) to 1 X 10(7) daltons and are similar to those in normal plasma. Normal cryosupernatant VIIIR:AG gave smaller molecular weights from 8 X 10(5) to 2 X 10(6) daltons. VIIIR:AG in the plasma and cryoprecipitate from 2 patients with classical vWd gave low precipitin peaks with molecular weights in normal range. VIIIR:AG from 2 patients with subgroup A variant which showed fast anodal migration on the conventional CIE, presented 3 peaks with molecular weight of 8 X 10(5) to 3 X 10(6) which are similar to those in normal cryosupernatant. VIIIR:AG from 2 patients with subgroup B variant which showed normal migration on the CIE, gave normal patterns through all fractions.

摘要

通过1.5%十二烷基硫酸钠聚丙烯酰胺凝胶电泳-交叉免疫电泳(SDS-PAGE-CIE)观察了不同类型血管性血友病(vWd)患者血浆及其组分、冷沉淀和冷上清液中血管性血友病因子抗原(VIIIR:AG)的模式。正常冷沉淀中的VIIIR:AG显示出几个沉淀素峰,其分子量范围为8×10⁵至1×10⁷道尔顿,与正常血浆中的相似。正常冷上清液中的VIIIR:AG分子量较小,为8×10⁵至2×10⁶道尔顿。2例典型vWd患者血浆和冷沉淀中的VIIIR:AG沉淀素峰较低,分子量在正常范围内。2例A亚组变异型患者的VIIIR:AG在传统CIE上显示向阳极快速迁移,呈现出3个分子量为8×10⁵至3×10⁶的峰,与正常冷上清液中的相似。2例B亚组变异型患者的VIIIR:AG在CIE上迁移正常,所有组分均呈现正常模式。

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