Lorenzo Hans-Kristian, Candelier Jean-Jacques
Inserm U1197, Interactions cellules souches-niches-physiologie, tumeurs et réparations tissulaires, Hôpital Paul Brousse, Bâtiment Lavoisier, 14, avenue Paul-Vaillant Couturier, 94800 Villejuif, France. - Université Paris-Saclay, Campus universitaire d'Orsay, 91 405 Orsay, France. - Service de néphrologie, CHU Bicêtre, 94270 Le Kremlin Bicêtre, France.
Inserm U1197, Interactions cellules souches-niches-physiologie, tumeurs et réparations tissulaires, Hôpital Paul Brousse, Bâtiment Lavoisier, 14, avenue Paul-Vaillant Couturier, 94800 Villejuif, France. - Université Paris-Saclay, Campus universitaire d'Orsay, 91 405 Orsay, France.
Med Sci (Paris). 2019 Aug-Sep;35(8-9):659-666. doi: 10.1051/medsci/2019128. Epub 2019 Sep 18.
The renal filtration is ensured by the kidney glomeruli selective for filtering the blood. The main actor of the glomerular filter is the podocyte whose interlaced pedicels bear protein complexes (nephrin, podocin, etc.) creating a molecular sieve (slit diaphragm) to achieve the filtration. Alterations of these podocytes lead to massive proteinuria, which characterizes the nephrotic syndrome. The idiopathic form is one of the most malignant and essentially comprises two entities: minimal change disease and focal segmental glomerulosclerosis. Many observations indicated that (1) immune cells are involved and that (2) there are several permeability factors in the blood that affect the morphology and function of podocytes (slit diaphragm with fractional foot processes fusion/effacement). Evidence for a permeability factor was chiefly derived from remission of proteinuria observed after implantation of a kidney with FSGS in healthy recipients or with other kidney diseases. Today, we are moving towards a multifactorial conception of the nephrotic syndrome where all these barely known factors could be associated according to a sequential kinetic mechanism that needs to be determined.
肾脏肾小球对血液进行选择性过滤，从而确保肾脏滤过功能。肾小球滤过的主要作用细胞是足细胞，其交错的足突带有蛋白质复合物（如nephrin、podocin等），形成分子筛（裂孔隔膜）以实现滤过。这些足细胞发生改变会导致大量蛋白尿，这是肾病综合征的特征。特发性形式是最严重的形式之一，主要包括两种类型：微小病变病和局灶节段性肾小球硬化症。许多观察结果表明：（1）免疫细胞参与其中；（2）血液中有多种通透性因子会影响足细胞的形态和功能（裂孔隔膜伴足突融合/消失）。通透性因子的证据主要来自于在健康受者或患有其他肾脏疾病的受者中植入患有局灶节段性肾小球硬化症的肾脏后蛋白尿缓解的情况。如今，我们正朝着肾病综合征的多因素概念发展，所有这些鲜为人知的因素可能会根据需要确定的顺序动力学机制相互关联。
