Molecular stratification of idiopathic nephrotic syndrome.,Nature Reviews Nephrology

当前位置： X-MOL 学术 › Nat. Rev. Nephrol. › 论文详情

Our official English website, www.x-mol.net, welcomes your feedback! (Note: you will need to create a separate account there.)

Molecular stratification of idiopathic nephrotic syndrome.
Nature Reviews Nephrology ( IF 28.6 ) Pub Date : 2019-10-25 , DOI: 10.1038/s41581-019-0217-5
Moin A Saleem _{1,

2}

Affiliation

Idiopathic nephrotic syndrome (INS) describes a group of pathologies of the renal glomerulus that result in the classic triad of heavy proteinuria, oedema and hypoalbuminaemia. The disease has historically been defined by evidence of distinctive histological changes in the absence of clinical evidence of a distinct pathological driver. However, the current classification is not based on any systematic mechanistic understanding of biological processes, and therefore current treatment regimens are broad, iterative and nonspecific. Over the past 20 years delineation of the underlying biology of the target cell in INS - the glomerular podocyte - has transformed our understanding of the mechanisms that contribute to breakdown of the glomerular filtration barrier and the development of INS. It is increasingly clear that nephrotic syndrome caused by monogenic mutations is distinct from immune-driven disease, which in some cases is mediated by circulating factors that target the podocyte. The combination of systems biology and bioinformatics approaches, together with powerful laboratory models and ever-growing patient registries has potential to identify disease 'signatures' that reflect the underlying molecular mechanism of INS on an individual basis. Understanding of such processes could lead to the development of targeted therapies.

中文翻译：

特发性肾病综合征的分子分层。

特发性肾病综合征 (INS) 描述了一组肾小球的病理，导致典型的重度蛋白尿、水肿和低白蛋白血症三联征。历史上，该疾病的定义是在没有明显病理驱动因素的临床证据的情况下，有明显的组织学变化证据。然而，目前的分类不是基于对生物过程的任何系统性机械理解，因此目前的治疗方案是广泛的、迭代的和非特异性的。在过去的 20 年中，对 INS 中靶细胞（肾小球足细胞）潜在生物学的描述改变了我们对导致肾小球滤过屏障破坏和 INS 发展的机制的理解。越来越清楚的是，由单基因突变引起的肾病综合征不同于免疫驱动的疾病，后者在某些情况下是由靶向足细胞的循环因子介导的。系统生物学和生物信息学方法的结合，加上强大的实验室模型和不断增长的患者登记，有可能在个体基础上识别反映 INS 潜在分子机制的疾病“特征”。了解这些过程可能会导致靶向治疗的发展。结合强大的实验室模型和不断增长的患者登记册，有可能识别出反映个体基础上 INS 潜在分子机制的疾病“特征”。了解这些过程可能会导致靶向治疗的发展。结合强大的实验室模型和不断增长的患者登记册，有可能识别出反映个体基础上 INS 潜在分子机制的疾病“特征”。了解这些过程可能会导致靶向治疗的发展。

更新日期：2019-10-25

点击分享查看原文

点击收藏

阅读更多本刊最新论文本刊介绍/投稿指南11