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系统性红斑狼疮患者替代途径及B因子溶血活性的评估:与替代途径调节蛋白的相关性

Evaluation of alternative pathway and factor B haemolytic activities in patients with systemic lupus erythematosus: correlations with the alternative pathway regulatory proteins.

作者信息

Aguado M T, Perrin L H, Ramirez R, Miescher P A, Lambert P H

出版信息

Clin Exp Immunol. 1980 Dec;42(3):495-505.

Abstract

The haemolytic activities of the complement alternative pathway and factor B were studied using a 51Cr-release assay in 77 sera from patients with systemic lupus erythematosus (SLE). These values were compared to those of several complement components and of the regulatory proteins beta H and C3b INA. There was a significant decrease in the alternative pathway activity, which correlated with a decrease in the haemolytic activity of factor B and with the activity of the classical pathway. There was a significant decrease in the level of one regulatory protein beta 1H, while the level of the other regulatory protein, C3b INA, was increased; however, there was a positive correlation between these two parameters. The results obtained suggest that the decreased activity of the alternative pathway observed in SLE reflects a consumption due to a triggering of the C3b amplification loop through the activation of the classical pathway. In one patient, an acquired deficiency of the capacity to activate the alternative pathway in the presence of inulin was observed.

摘要

利用51Cr释放试验,对77例系统性红斑狼疮(SLE)患者血清中的补体替代途径和B因子的溶血活性进行了研究。将这些值与几种补体成分以及调节蛋白βH和C3bINA的值进行了比较。替代途径活性显著降低,这与B因子溶血活性降低以及经典途径活性降低相关。一种调节蛋白β1H水平显著降低,而另一种调节蛋白C3bINA水平升高;然而,这两个参数之间存在正相关。所得结果表明,SLE中观察到的替代途径活性降低反映了由于经典途径激活触发C3b放大环而导致的消耗。在一名患者中,观察到在存在菊粉的情况下激活替代途径的能力获得性缺陷。

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