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C4结合蛋白在系统性红斑狼疮患者经典途径C3转化酶调节中的相对重要性

Relative importance of C4 binding protein in the modulation of the classical pathway C3 convertase in patients with systemic lupus erythematosus.

作者信息

Daha M R, Hazevoet H M, Hermans J, van Es L A, Cats A

出版信息

Clin Exp Immunol. 1983 Oct;54(1):248-52.

Abstract

Serum concentrations of C1q, C4, C4 binding protein (C4bp), C3 and C2 haemolytic activity have been measured in 110 samples from 20 patients with systemic lupus erythematosus (SLE). Significant reductions in comparison to normal levels were found in the mean serum concentrations of C4, C3 and C4bp as well as C2 haemolytic activities. For patients serum concentrations of C4 correlated with C2 haemolytic activities (r = 0.91) and C4bp (r = 0.79); the C2 haemolytic levels correlated with the concentration of C4b (r = 0.72). It is concluded that serum concentrations of the complement components C4 and C2, which are the constituents of the classical pathway C3 convertase, are regulated by C4bp in vivo. Further metabolic studies are required to determine the causes of decreased serum concentrations of C4bp in patients with SLE.

摘要

已对20例系统性红斑狼疮(SLE)患者的110份样本进行了C1q、C4、C4结合蛋白(C4bp)、C3和C2溶血活性的血清浓度测定。与正常水平相比,C4、C3和C4bp的平均血清浓度以及C2溶血活性均显著降低。对于患者,血清C4浓度与C2溶血活性(r = 0.91)和C4bp(r = 0.79)相关;C2溶血水平与C4b浓度相关(r = 0.72)。得出结论,经典途径C3转化酶的组成成分补体成分C4和C2的血清浓度在体内受C4bp调节。需要进一步的代谢研究来确定SLE患者血清C4bp浓度降低的原因。

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