Department of Pediatrics, Yale University School of Medicine, New Haven, CT, 06520, USA.
Department of Internal Medicine, Yale University School of Medicine, New Haven, CT, 06520, USA.
Sci Rep. 2017 Aug 29;7(1):9833. doi: 10.1038/s41598-017-10490-z.
Nephrotic syndrome is a common disorder in adults and children whose etiology is largely unknown. Glucocorticoids remain the mainstay of therapy in most cases, though their mechanism of action remains poorly understood. Emerging evidence suggests that immunomodulatory therapies used in nephrotic syndrome directly target the podocytes. To study how steroids directly affect the podocytes in the treatment of proteinuria, we created a mouse model with podocyte-specific deletion of the glucocorticoid receptor. The podocyte-specific glucocorticoid receptor (GR) knockout mice had similar renal function and protein excretion compared to wild type. However, after glomerular injury induced by either LPS or nephrotoxic serum, the podocyte GR knockout mice demonstrated worsened proteinuria compared to wild type. Ultrastructural examination of podocytes confirmed more robust foot process effacement in the knockout animals. Expression of several key slit diaphragm protein was down regulated in pGR KO mice. Primary podocytes isolated from wild type and podocyte GR knockout mice showed similar actin stress fiber staining patterns in unstimulated conditions. Yet, when exposed to LPS, GR knockout podocytes demonstrated fewer stress fibers and impaired migration compared to wild type podocytes. We conclude that the podocyte glucocorticoid receptor is important for limiting proteinuria in settings of podocyte injury.
肾病综合征是一种常见的成人和儿童疾病,其病因在很大程度上尚不清楚。在大多数情况下,糖皮质激素仍然是治疗的主要方法,尽管其作用机制仍不清楚。新出现的证据表明,肾病综合征中使用的免疫调节疗法直接针对足细胞。为了研究类固醇在治疗蛋白尿时如何直接影响足细胞,我们创建了一种足细胞特异性缺失糖皮质激素受体的小鼠模型。与野生型相比,足细胞特异性糖皮质激素受体 (GR) 敲除小鼠具有相似的肾功能和蛋白尿排泄。然而,在 LPS 或肾毒性血清诱导的肾小球损伤后,与野生型相比,足细胞 GR 敲除小鼠表现出更严重的蛋白尿。足细胞的超微结构检查证实,敲除动物的足突融合更明显。几个关键的裂孔隔膜蛋白的表达在 pGR KO 小鼠中下调。从野生型和足细胞 GR 敲除小鼠分离的原代足细胞在未刺激条件下显示出相似的肌动蛋白应激纤维染色模式。然而,当暴露于 LPS 时,与野生型足细胞相比,GR 敲除足细胞显示出更少的应激纤维和受损的迁移。我们的结论是,足细胞糖皮质激素受体对于限制足细胞损伤时的蛋白尿是重要的。