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膜增生性肾小球肾炎中的经典补体途径激活

Classical complement pathway activation in membranoproliferative glomerulonephritis.

作者信息

Ooi Y M, Vallota E H, West C D

出版信息

Kidney Int. 1976 Jan;9(1):46-53. doi: 10.1038/ki.1976.6.

DOI:10.1038/ki.1976.6
PMID:781380
Abstract

Levels of components of the classical and alternative complement pathways and the activity of the C3 nephritic factor (C3NeF) were measured in serum specimens from patients with type I (subendothelial deposits) and type II (intramembranous dense deposits) membranoproliferative glomerulonephritis (MPGN) and the results compared with the levels in normal subjects. Although C3 and C5 concentrations were comparably depressed in type I and type II, the levels of Clq and C4 were lower in type I and the correlation coefficients between Clq vs. C4 and C4 vs. C3 were higher than in type II. The profile was highly suggestive of classical pathway activation. In contrast, in type II MPGN, factor B levels were lower and C3NeF activity was more frequently detectable and higher. A feature of interest was that type I, as compared to type II, was characterized by significantly lower serum concentrations of properdin, higher and more significant correlation coefficients between serum properdin concentrations and those of Clq, C4 and C3, and consistent glomerular deposition of properdin. The involvement of properdin in type I may reflect recruitment of a pathway resembling the C1 bypass mechanism, said to involve C1, properdin, factor B and C3-9.

摘要

在I型(内皮下沉积物)和II型(膜内致密沉积物)膜增生性肾小球肾炎(MPGN)患者的血清标本中,检测了经典补体途径和替代补体途径各成分的水平以及C3肾炎因子(C3NeF)的活性,并将结果与正常受试者的水平进行比较。尽管I型和II型中C3和C5浓度均有相当程度的降低,但I型中Clq和C4水平较低,且Clq与C4以及C4与C3之间的相关系数高于II型。该结果强烈提示经典途径被激活。相比之下,在II型MPGN中,B因子水平较低,C3NeF活性更常被检测到且更高。一个有趣的特点是,与II型相比,I型的特征是血清备解素浓度显著降低,血清备解素浓度与Clq、C4和C3浓度之间的相关系数更高且更显著,并且备解素在肾小球中持续沉积。备解素在I型中的参与可能反映了一种类似于C1旁路机制途径的募集,据说该机制涉及C1、备解素、B因子和C3 - 9。

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Classical complement pathway activation in membranoproliferative glomerulonephritis.膜增生性肾小球肾炎中的经典补体途径激活
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