The complex multimeric composition of factor VIII/von Willebrand factor
The complex multimeric composition of factor VIII/von Willebrand factor
复制标题
因子 VIII/冯维勒布兰德因子的复杂多聚体组成
DOI：
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发表时间：
1981
期刊：
影响因子：
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通讯作者：
T. Zimmerman
中科院分区：
文献类型：
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作者：
Z. Ruggeri;T. Zimmerman
我们已经分析了多聚体结构的因子VIII/血管性血友病因子在血浆中的十二烷基硫酸钠电泳使用不同的孔隙率和不连续的缓冲液系统的凝胶。通过与125 I标记的亲和纯化抗体反应和随后的放射自显影鉴定因子VIII/von Willebrand因子条带。在1%琼脂糖凝胶中，正常血浆显示出一系列清晰的寡聚体。然而，将琼脂糖浓度增加到2.0%或使用0.8%琼脂糖-1.75%丙烯酰胺的混合物显示出插入在主要条带之间的两条强度较低的条带。当凝胶中的丙烯酰胺浓度增加到2.5%时，具有比IgM和纤连蛋白更快的迁移率的条带现在是明显的。与IIB型、I型和正常相比，IIA型血管性血友病不仅没有较大的多聚体，而且几个新鉴定的条带相对增加。这些研究表明，因子VII/血管性血友病因子在IIA型血管性血友病中的结构不同于其他形式的疾病。他们还表明，因子VII/血管性血友病因子的多聚体组合物是更复杂的比可以解释的简单的线性聚合的一个单一的原聚体。
We have analyzed the multimeric structure of factor VIII/von Willebrand factor in plasma by sodium dodecyl sulfate electrophoresis using gels of varying porosity and a discontinuous buffer system. Factor VIII/von Willebrand factor bands were identified by reaction with 125I-labeled affinity-purified antibody and subsequent autoradiography. In 1% agarose gels, normal plasma displayed a series of sharply defined oligomers. However, increasing the agarose concentration to 2.0% or utilizing mixtures of 0.8% agarose--1.75% acrylamide revealed two bands of lesser intensity interposed between the major bands. When the acrylamide concentration in the gels was increased to 2.5%, bands with a faster mobility than IgM and fibronectin were now evident. Type IIA von Willebrand's disease showed not only an absence of the larger multimers but also a relative increase in several of the newly identified bands as compared to type IIB, type I, and normal. These studies suggest that factor VII/von Willebrand factor in IIA von Willebrand's disease is structurally different from that in other forms of the disorder. They also indicate that the multimeric composition of factor VII/von Willebrand factor is more complex than can be explained by simple linear polymerization of a single protomer.