EFFECT OF VON WILLEBRAND FACTOR ON THE ACTIVITY OF FACTOR VIII

血管性血友病因子对因子 VIII 活性的影响

• 批准号: 3811121
• 负责人: WILLIAM FRICKE
• 金额: --
• 依托单位: DIVISION OF BASIC SCIENCES - NCI
• 依托单位国家: 美国
• 资助国家: 美国
• 起止时间: 至
• 项目状态: 未结题
• 来源: https://reporter.nih.gov/project-details/3811121
• 关键词: blocking antibody; coagulation factor VIII; immunoelectrophoresis; immunologic assay /test; von Willebrand factor

Investigation of interaction between factor VIII (fVIII) and von Willebrand factor (vWF) has been undertaken along several lines. First, the ability of vWF to block the interaction of factor VIII with antibodies to factor VIII has been studied by using the Bethesda assay. Inhibition of factor VIII activity by the antibodies is greatest when factor VIII preparations containing little or no vWF are tested (approx. 12,000 BU), decreases to about 5,000 BU for ratios of fVIII:vWF seen in plasma, and to 3500 for preparations with 3 times more vWF than fVIII. Ongoing work is intended to determine if this vWF effect is due to a vWF effect on the assay or represents blocking of the antibody by vWF. Second, the effect of vWF on factor VIII assays is being studied. Data generated thus far indicate that the vWF:Ag level must be at least 50% or normal for full expression of FVIII:C. And, third, the association of FVIII:Ag with individual vWF multimers is being investigated by SDS-agarose electrophoresis using radiolabelled anti-FVIII antibodies. Preliminary data indicate that FVIII is found in association with the small and intermediate size multimers.

因子VIII（fVIII）与von Willebrand之间相互作用的研究 已经沿着沿着几条路线进行了vWF因子的研究。 第一，能力 vWF阻断因子VIII与因子抗体的相互作用 VIII已经通过使用Bethesda测定法进行了研究。 抑制因子 当凝血因子VIII制剂被抗体激活时， 含有很少或没有vWF的测试（约100 μ g/ml）。12，000 BU），降至 血浆中fVIII：vWF比值约为5，000 BU， vWF是fVIII的3倍。 正在进行的工作旨在 确定该vWF效应是否是由于vWF对试验的影响，或 代表vWF阻断抗体。 第二，vWF对 正在研究因子VIII测定法。 迄今为止生成的数据表明， vWF：Ag水平必须至少为50%或正常，以完全表达 FVIII：C. 第三，FVIII：Ag与个体vWF的相关性 使用SDS-琼脂糖电泳研究多聚体， 放射性标记的抗FVIII抗体。 初步数据表明，FVIII 发现与小和中等大小的多聚体相关。