The parietal epithelial cell is crucially involved in human idiopathic focal segmental glomerulosclerosis
The parietal epithelial cell is crucially involved in human idiopathic focal segmental glomerulosclerosis
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DOI：
10.1111/j.1523-1755.2005.00568.x
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发表时间：
2005-10-01
影响因子：
19.6
通讯作者：
Wetzels, J
中科院分区：
文献类型：
--
作者：
Dijkman, H;Smeets, B;Wetzels, J
背景。局灶节段性肾小球硬化症（FSGS）是人类肾活检中最常见的肾小球损伤模式之一。上皮增生在 FSGS 中尤为突出，归因于足细胞的去分化和增殖。根据对 FSGS 小鼠模型的观察，我们指出了壁上皮细胞 (PEC) 的作用。在本研究中，我们研究了 PEC 和足细胞在人类特发性 FSGS 中的相对作用。方法。我们通过连续切片、标记物分析和肾小球三维重建，对复发性特发性 FSGS 患者的病变进行了详细研究。我们研究了足细胞、PEC、系膜细胞、内皮细胞和肌成纤维细胞标记物的表达。我们还观察了沉积的细胞外基质 (ECM) 的增殖和组成。结果。我们发现 FSGS 病变中的增殖上皮细胞足细胞和巨噬细胞标记物呈阴性，但 PEC 标记物染色。这些细胞沉积的基质的组成与鲍曼囊相同。结论。我们的研究表明 PEC 在 FSGS 病变的发病机制中发挥着至关重要的作用。
Background. Focal segmental glomerulosclerosis (FSGS) is one of the most common patterns of glomerular injury encountered in human renal biopsies. Epithelial hyperplasia, which can be prominent in FSGS, has been attributed to dedifferentiation and proliferation of podocytes. Based on observations in a mouse model of FSGS, we pointed to the role of parietal epithelial cells (PECs). In the present study we investigated the relative role of PECs and podocytes in human idiopathic FSGS.Methods. We performed a detailed study of lesions from a patient with recurrent idiopathic FSGS by serial sectioning, marker analysis and three-dimensional reconstruction of glomeruli. We have studied the expression of markers for podocytes, PECs, mesangial cells, endothelium, and myofibroblasts. We also looked at proliferation and composition of the deposited extracellular matrix (ECM).Results. We found that proliferating epithelial cells in FSGS lesions are negative for podocyte and macrophage markers, but stain for PEC markers. The composition of the matrix deposited by these cells is identical to Bowman's capsule.Conclusion. Our study demonstrates that PECs are crucially involved in the pathogenesis of FSGS lesions.