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在Ⅰ型和Ⅲ型膜增生性肾小球肾炎中,一种依赖备解素的肾炎因子缓慢激活C3、C5和C9。

A properdin dependent nephritic factor slowly activating C3, C5, and C9 in membranoproliferative glomerulonephritis, types I and III.

作者信息

Clardy C W, Forristal J, Strife C F, West C D

机构信息

Children's Hospital Research Foundation, Cincinnati, Ohio 45229-2899.

出版信息

Clin Immunol Immunopathol. 1989 Mar;50(3):333-47. doi: 10.1016/0090-1229(89)90141-4.

Abstract

The IgG fraction of serum from patients with membranoproliferative glomerulonephritis (MPGN) types I and III was found to contain a nephritic factor (NFI/III) which differed from that usually present in MPGN type II and partial lipodystrophy (NFII) in that it converts C5 and C9 as well as C3, is dependent on properdin for its activity, and requires an incubation period of several hours rather than 30 min for its demonstration. C3 conversion occurred in the absence of an intact classical pathway. This nephritic factor was found in patients with reduced serum levels of terminal components and its activity, like that of the nephritic factor in MPGN type II, correlated with the serum C3 level indicating that these nephritic factors play a large role in producing hypocomplementemia. Although potentially nephritogenic because of its ability to activate the terminal pathway, the presence of this nephritic factor did not clearly correlate with clinical course.

摘要

在I型和III型膜增生性肾小球肾炎(MPGN)患者的血清IgG组分中发现了一种肾炎因子(NFI/III),它与通常存在于II型MPGN和部分脂肪营养不良(NFII)中的肾炎因子不同,因为它能转化C5、C9以及C3,其活性依赖备解素,且需要数小时的孵育期才能显现,而不是30分钟。在经典途径完整的情况下也会发生C3转化。在血清终末成分水平降低的患者中发现了这种肾炎因子,其活性与II型MPGN中的肾炎因子一样,与血清C3水平相关,表明这些肾炎因子在导致低补体血症中起很大作用。尽管由于其激活终末途径的能力可能具有致肾炎性,但这种肾炎因子的存在与临床病程并没有明显的相关性。

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