Glomeruli of Dense Deposit Disease contain components of the alternative and terminal complement pathway.
Glomeruli of Dense Deposit Disease contain components of the alternative and terminal complement pathway.
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DOI：
10.1038/ki.2008.657
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发表时间：
2009-05
影响因子：
19.6
通讯作者：
Smith, Richard J. H.
中科院分区：
文献类型：
--
作者：
Sethi, Sanjeev;Gamez, Jeffrey D.;Vrana, Julie A.;Theis, Jason D.;Bergen, H. Robert, III;Zipfel, Peter F.;Dogan, Ahmet;Smith, Richard J. H.
关键词：
致密存款病（DDD），或膜增生性肾小球肾炎II型，是一种罕见的肾脏疾病，其特征是致密沉积物在系膜和沿着肾小球基底膜，可以通过电子显微镜观察到。虽然这些存款含有补体因子C3，免疫荧光显微镜测定，其精确的组成仍然未知。为了解决这个问题，我们使用质谱法来鉴定从8例确诊DDD病例的石蜡包埋组织中分离的激光显微切割肾小球中的蛋白质。与5例对照组相比，我们发现DDD患者的所有肾小球均含有旁路途径和末端补体复合物的成分。因子C9是均匀存在的，以及两个液相调节剂的末端补体复合物clusterin和玻连蛋白。相反，在9例免疫复合物介导的膜增生性肾小球肾炎患者中，肾小球样本主要含有免疫球蛋白和补体因子C3和C4。我们的研究表明，除了替代途径的液相失调，终末补体复合物的可溶性成分有助于DDD中发现的肾小球病变。
Dense Deposit Disease (DDD), or membranoproliferative glomerulonephritis type II, is a rare renal disease characterized by dense deposits in the mesangium and along the glomerular basement membranes that can be seen by electron microscopy. Although these deposits contain complement factor C3, as determined by immunofluorescence microscopy, their precise composition remains unknown. To address this question, we used mass spectrometry to identify the proteins in laser microdissected glomeruli isolated from paraffin-embedded tissue of eight confirmed cases of DDD. Compared to glomeruli from five control patients, we found that all of the glomeruli from patients with DDD contain components of the alternative pathway and terminal complement complex. Factor C9 was uniformly present as well as the two fluid-phase regulators of terminal complement complex clusterin and vitronectin. In contrast, in nine patients with immune complex–mediated membranoproliferative glomerulonephritis, glomerular samples contained mainly immunoglobulins and complement factors C3 and C4. Our study shows that in addition to fluid-phase dysregulation of the alternative pathway, soluble components of the terminal complement complex contribute to glomerular lesions found in DDD.
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影响因子：
4.4
作者：
Schwertz, R;Rother, U;Kirschfink, M
通讯作者：
Kirschfink, M
影响因子：
3.6
作者：
Montes, Tamara;Goicoechea de Jorge, Elena;Rodriguez de Cordoba, Santiago
通讯作者：
Rodriguez de Cordoba, Santiago
DOI：
10.1073/pnas.0601094103
发表时间：
2006-06-20
影响因子：
11.1
作者：
Pickering, M. C.;Warren, J.;Botto, M.
通讯作者：
Botto, M.
影响因子：
4.4
作者：
Ferreira, Viviana P.;Herbert, Andrew P.;Pangburn, Michael K.
通讯作者：
Pangburn, Michael K.
影响因子：
13.6
作者：
Smith, Richard J. H.;Alexander, Jessy;Zipfel, Peter F.
通讯作者：
Zipfel, Peter F.