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致密沉积物病的免疫荧光研究。铁轨样结构和系膜环的存在。

Immunofluorescence studies of dense deposit disease. The presence of railroad tracks and mesangial rings.

作者信息

Kim Y, Vernier R L, Fish A J, Michael A F

出版信息

Lab Invest. 1979 Apr;40(4):474-80.

PMID:372673
Abstract

Immunopathologic analysis was carried out on renal tissue from patients with membranoproliferative glomerulonephritis, nine with type I and nine with type II (dense deposit disease). A specific finding in all patients with type II, but not type I, was the presence of C3 along the margin but not within the central portion of dense deposit material in the glomerular basement membrane giving a double linear appearance (railroad tracks); C3 was also present within the mesangium, outlining numerous circular structures (mesangial rings). By phase contrast, electron microscopy, and dual label fluorescence studies, the railroad tracks and mesangial rings were shown to outline dense deposit material. Mesangial rings contained properdin (four of nine patients); railroad tracks contained properdin (five of nine patients) and C4 (four of nine patients); no other complement components or immunoglobulins were present. Studies using rhodamine-conjugated rabbit antibody to human glomerular basement membrane demonstrated no reactivity with the dense deposit material itself. In type I and to a lesser extent in type II, granular deposits of C3, C4, properdin, IgM, and IgG were present along the glomerular basement membrane, suggesting that immune complexes play a role in both diseases. Dense deposit transformation of the glomerular basement membrane may be a consequence of a highly specific injury.

摘要

对膜增生性肾小球肾炎患者的肾组织进行了免疫病理学分析,其中I型9例,II型(致密物沉积病)9例。II型患者(而非I型患者)的一个特异性发现是,在肾小球基底膜致密物沉积物质的边缘而非中央部分有C3存在,呈现出双线外观(铁轨样);C3也存在于系膜中,勾勒出许多圆形结构(系膜环)。通过相差显微镜、电子显微镜和双标记荧光研究发现,铁轨样结构和系膜环勾勒出致密物沉积物质。系膜环含有备解素(9例患者中有4例);铁轨样结构含有备解素(9例患者中有5例)和C4(9例患者中有4例);不存在其他补体成分或免疫球蛋白。使用罗丹明偶联的抗人肾小球基底膜兔抗体进行的研究表明,该抗体与致密物沉积物质本身无反应性。在I型以及程度较轻的II型中,沿肾小球基底膜存在C3、C4、备解素、IgM和IgG的颗粒状沉积物,提示免疫复合物在这两种疾病中均起作用。肾小球基底膜的致密物沉积转变可能是一种高度特异性损伤的结果。

相似文献

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Immunofluorescence studies of dense deposit disease. The presence of railroad tracks and mesangial rings.致密沉积物病的免疫荧光研究。铁轨样结构和系膜环的存在。
Lab Invest. 1979 Apr;40(4):474-80.
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Membranoproliferative glomerulonephritis. Localization of early components of complement in glomerular deposits.膜增生性肾小球肾炎。补体早期成分在肾小球沉积物中的定位。
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备解素和因子 H:补体旁路途径的“跷跷板”上的对立玩家。
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Factor I is required for the development of membranoproliferative glomerulonephritis in factor H-deficient mice.在缺乏补体因子H的小鼠中,补体因子I是膜增生性肾小球肾炎发生所必需的。
J Clin Invest. 2008 Feb;118(2):608-18. doi: 10.1172/JCI32525.
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J Am Soc Nephrol. 2007 Sep;18(9):2447-56. doi: 10.1681/ASN.2007030356. Epub 2007 Aug 5.
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