Expression of nephrin, podocin, α-actinin, and WT1 in children with nephrotic syndrome
Expression of nephrin, podocin, α-actinin, and WT1 in children with nephrotic syndrome
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DOI：
10.1007/s00467-003-1240-z
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发表时间：
2003-11-01
影响因子：
3
通讯作者：
Yang, JY
中科院分区：
文献类型：
--
作者：
Guan, N;Ding, J;Yang, JY
近年来，研究发现位于膈缝处并由足细胞表达的nephrin、podocin、α - actitin和WT1是先天性/家族性肾病综合征（NS）的病因，但它们在获得性NS中的作用尚不清楚。我们研究了它们在NS中的表达，目的是揭示它们在蛋白尿发生中的可能作用。采用免疫荧光、共聚焦显微镜和图像分析方法研究了19例原发性NS患儿、9例分离性血尿患儿和9例对照患儿的表达和分布。所有NS患儿均表现为大量蛋白尿和足突消失。血尿组未见蛋白尿和足突消失。与对照组相比，NS组podocin表达显著降低（86.66+/-22.74）（P=0.014）。此外，我们还发现肾病患儿的nephrin、podocin和α - actitin的分布模式发生了变化。综上所述，NS患儿中podocin表达显著降低，nephrin、podocin、α - actiin分布异常。在孤立性血尿患儿中未发现差异，提示这些分子参与了原发性NS中蛋白尿的发展。
Recently, nephrin, podocin, alpha-actinin, and WT1, which are located at the slit diaphragm and expressed by the podocyte, were found to be causative in congenital/familial nephrotic syndrome (NS), but their role in acquired NS remains unclear. We studied their expression in NS with the aim of disclosing their possible role in the development of proteinuria. Immunofluorescence, confocal microscopy, and image analysis were used to study the expression and the distribution in 19 children with primary NS, 9 with isolated hematuria, and 9 controls. All the children with NS presented with heavy proteinuria and foot process effacement was identified by electron microscopy. No proteinuria and foot process effacement was seen in the group with hematuria. A dramatic decrease of podocin expression was found in NS (86.66+/-22.74) compared with control groups (P=0.014). Furthermore, we also found the pattern of distribution of nephrin, podocin, and alpha-actinin changed in children with NS. In conclusion, a dramatic decrease of podocin expression and abnormal distribution of nephrin, podocin, and alpha-actinin were found in children with NS. No differences were found in children with isolated hematuria, suggesting involvement of these molecules in the development of proteinuria in primary NS.