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Expression of nephrin, podocin, α-actinin, and WT1 in children with nephrotic syndrome

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Abstract

Recently, nephrin, podocin, α-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 α-actinin changed in children with NS. In conclusion, a dramatic decrease of podocin expression and abnormal distribution of nephrin, podocin, and α-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.

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Acknowledgements.

This study was supported by National Nature Science Foundation of China (30170992). We are grateful to Professor Karl Tryggvason (Sweden) and Corinne Antignac (France) for their kind gift of antibodies. We also thank Jianping Huang, Yong Yao, Huijie Xiao, and Jingcheng Liu for their support of this project.

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Authors and Affiliations

  1. Department of Pediatrics, Peking University First Hospital, Peking, China

    Na Guan, Jie Ding, Jingjing Zhang & Jiyun Yang

  2. Department of Pediatrics, Peking University First Hospital, No.1 Xi An Men Da Jie, 100034, Beijing, P. R. China

    Jie Ding

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  1. Na Guan
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Correspondence to Jie Ding.

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Guan, N., Ding, J., Zhang, J. et al. Expression of nephrin, podocin, α-actinin, and WT1 in children with nephrotic syndrome. Pediatr Nephrol 18, 1122–1127 (2003). https://doi.org/10.1007/s00467-003-1240-z

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  • DOI: https://doi.org/10.1007/s00467-003-1240-z

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