New insights into human minimal change disease: lessons from animal models.
New insights into human minimal change disease: lessons from animal models.
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DOI：
10.1053/j.ajkd.2011.07.024
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发表时间：
2012-02
期刊：
影响因子：
--
通讯作者：
Macé C
中科院分区：
文献类型：
--
作者：
Chugh SS;Clement LC;Macé C
微小改变病（MCD）被认为是肾病综合征最简单的形式，其发病机制一直是肾脏疾病未解之谜之一。在这篇综述中，最近具有里程碑意义的研究导致MCD的解开进行了讨论。今年早些时候发表的一项研究解释了MCD主要临床和形态学变化的分子基础。MCD足细胞中血管生成素样4 （ANGPTL4）的过量产生导致ANGPTL4与肾小球基底膜（GBM）结合，肾范围选择性蛋白尿的发展，足突弥漫性消失和GBM电荷的损失，但与光镜下肾小球和小管间质室的变化无关。ANGPTL4诱导蛋白尿的能力至少部分与唾液酸残基的缺乏有关，因为口服补充唾液酸前体n -乙酰- d -甘露胺可改善足细胞分泌ANGPTL4的唾液化，并显著减少蛋白尿。本文还讨论了MCD的动物模型、潜在生物标志物的最新进展以及可能引发肾小球改变的上游因素的研究。综上所述，最近对MCD的了解进展可能会在不久的将来影响MCD的诊断和治疗。
The pathogenesis of minimal change disease (MCD), considered to be the simplest form of nephrotic syndrome, has been one of the major unsolved mysteries in kidney disease. In this review, recent landmark studies that have led to the unraveling of MCD are discussed. A study published earlier this year now explains the molecular basis of major clinical and morphological changes in MCD. The overproduction of angiopoietin-like 4 (ANGPTL4) in podocytes in MCD causes binding of ANGPTL4 to the glomerular basement membrane (GBM), development of nephrotic-range selective proteinuria, diffuse effacement of foot processes, and loss of GBM charge, but is not associated with changes shown by light microscopy in the glomerular and tubulointerstitial compartments. At least some of this ability of ANGPTL4 to induce proteinuria is linked to a deficiency of sialic acid residues, since oral supplementation with sialic acid precursor N-acetyl-D-mannosamine improves sialylation of podocyte secreted ANGPTL4 and significantly reduces proteinuria. Animal models of MCD, recent advances in potential biomarkers, and studies on upstream factors that may initiate glomerular changes are also discussed. In summary, recent progress in understanding MCD is likely to influence the diagnosis and treatment of MCD in the near future.
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影响因子：
4.8
作者：
Luchansky, SJ;Yarema, KJ;Bertozzi, CR
通讯作者：
Bertozzi, CR
DOI：
10.1084/jem.194.1.13
发表时间：
2001-07-02
期刊：
The Journal of experimental medicine
影响因子：
--
作者：
Doyonnas R;Kershaw DB;Duhme C;Merkens H;Chelliah S;Graf T;McNagny KM
通讯作者：
McNagny KM
影响因子：
20.1
作者：
Cazes, Aurelie;Galaup, Ariane;Monnot, Catherine
通讯作者：
Monnot, Catherine
影响因子：
19.6
作者：
Chugh, S;Yuan, HP;Salant, DJ
通讯作者：
Salant, DJ
影响因子：
13.6
作者：
Lai, Kin-Wai;Wei, Chang-Li;Yap, Hui-Kim
通讯作者：
Yap, Hui-Kim