Factor I and factor H deficiency in renal diseases: similar defects in the fluid phase have a different outcome at the surface of the glomerular basement membrane.
Factor I and factor H deficiency in renal diseases: similar defects in the fluid phase have a different outcome at the surface of the glomerular basement membrane.
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肾脏疾病中因子 I 和因子 H 缺乏：相似的液相缺陷在肾小球基底膜表面会产生不同的结果。
DOI：
10.1093/ndt/gfn652
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发表时间：
2009
期刊：
影响因子：
--
通讯作者：
Skerka,Christine
中科院分区：
文献类型：
--
作者：
Zipfel,PeterF;Smith,RichardJH;Skerka,Christine
II型膜增生性肾小球肾炎（MPGN II），也称为致密存款病（DDD），与循环中和生物表面上补体替代途径的失调相关，其导致补体活化产物沿着肾小球基底膜（GBM）的局部沉积。由于因子I和因子H都控制溶液中的替代补体激活，并且由于因子H的缺乏导致MPGN，Matthew Pickering及其同事探索了因子I的缺乏可能导致某种相似的肾脏表型的可能性。他们产生了因子I缺陷小鼠，并将这些动物的肾脏疾病与因子H缺陷小鼠和因子H和因子I联合缺陷的动物中观察到的肾脏疾病进行了比较[1]。因子H缺陷小鼠的肾脏疾病特征为肾小球毛细血管壁上的C3沉积、系膜细胞过多、外周毛细血管袢增厚和GBM双重轮廓，与MPGN II的诊断完全一致[2]。在因子I缺陷小鼠中，尽管肾小球变化包括细胞过多、系膜扩张和毛细血管壁增厚，但不存在MPGN II的光镜特征，包括毛细血管壁双重轮廓，并且肾小球C3染色仅分布于系膜，与因子I缺陷小鼠中的线性毛细血管壁染色模式形成鲜明对比。
Membranoproliferative glomerulonephritis type II (MPGN II), also termed dense deposit disease (DDD), is associated with dysregulation of the alternative pathway of complement in the circulation and on biosurfaces that leads to the local deposition of complement activation products along the glomerular basement membrane (GBM). As both Factor I and Factor H control alternative complement activation in a solution and as deficiency of Factor H leads to MPGN, Matthew Pickering and colleagues explored the possibility that the deficiency of Factor I might lead to a somewhat similar renal phenotype. They generated Factor I-deficient mice and compared the renal disease in these animals to that observed in Factor H-deficient mice and animals with combined Factor H and Factor I deficiency [1]. Renal disease in Factor H-deficient mice is characterized by C3 deposition on glomerular capillary walls, mesangial hypercellularity, peripheral capillary loop thickening and double contouring of the GBM, entirely consistent with the diagnosis of MPGN II [2]. In Factor I-deficient mice, although glomerular changes included hypercellularity, mesangial expansion and capillary wall thickening, light microscopic features of MPGN II, including capillary wall double contours, were absent and glomerular C3 staining was only mesangial in distribution, in striking contrast to the linear capillary wall staining pattern in the Factor
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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：
13.6
作者：
Smith, Richard J. H.;Alexander, Jessy;Zipfel, Peter F.
通讯作者：
Zipfel, Peter F.
影响因子：
13.6
作者：
Appel, GB;Cook, HT;Zipfel, PF
通讯作者：
Zipfel, PF
影响因子：
4.4
作者：
T. Jokiranta;A. Solomon;M. K. Pangburn;P. Zipfel;S. Meri
通讯作者：
T. Jokiranta;A. Solomon;M. K. Pangburn;P. Zipfel;S. Meri
C3 肾炎因子 (C3NeF)：稳定液相和细胞结合旁路途径转化酶。
影响因子：
4.4
作者：
M. Daha;D. Fearon;K. Austen
通讯作者：
K. Austen