Ramos J C, Maeda N Y, Lopes A A
Fundação Pró-Sangue Hemocentro de São Paulo, Brasil.
Braz J Med Biol Res. 1995 Aug;28(8):853-7.
We evaluated the correlation between decreased biological activity and abnormalities in the multimeric structure of plasma von Willebrand factor (vWF) in 27 pulmonary hypertensive patients (median age, 21 years). The biological activity of vWF was measured by the ristocetin cofactor assay and its multimeric structure was assessed by Western immunoblotting after SDS-agarose gel electrophoresis. In spite of high antigenic activity of vWF in plasma (139 +/- 65 vs 91 +/- 27% in controls, P = 0.003), the biological activity expressed as a percent of the control value was decreased in pulmonary hypertensive patients (60-88% activity, 95% CI for the mean). High molecular weight multimers (biologically active forms) were absent in patients and there was a significant increase in the concentration of low molecular weight polymers in comparison with normals (56 +/- 12 and 35 +/- 12% of total multimer density, respectively, P < 0.001). Multimeric abnormalities were positively correlated with plasma vWF levels (r = 0.51, P = 0.007) and negatively correlated with vWF biological activity (r = -0.54, P = 0.004). Thus, decreased biological function is related to abnormalities in the multimeric structure of vWF, possibly reflecting extensive endothelial dysfunction in pulmonary hypertension.
我们评估了27例肺动脉高压患者（中位年龄21岁）血浆血管性血友病因子（vWF）生物活性降低与多聚体结构异常之间的相关性。通过瑞斯托霉素辅因子试验测定vWF的生物活性，并在SDS-琼脂糖凝胶电泳后通过Western免疫印迹法评估其多聚体结构。尽管血浆中vWF的抗原活性较高（139±65 vs对照组的91±27%，P = 0.003），但肺动脉高压患者中以对照值百分比表示的生物活性降低（活性为60 - 88%，均值的95%CI）。患者中不存在高分子量多聚体（生物活性形式），与正常相比，低分子量聚合物的浓度显著增加（分别占总多聚体密度的56±12和35±12%，P < 0.001）。多聚体异常与血浆vWF水平呈正相关（r = 0.51，P = 0.007），与vWF生物活性呈负相关（r = -0.54，P = 0.004）。因此，生物功能降低与vWF多聚体结构异常有关，这可能反映了肺动脉高压中广泛的内皮功能障碍。
