Nephrin and Neph-family proteins [Neph1–3 (nephrin-like 1–3)] belong to the immunoglobulin superfamily of cell-adhesion receptors and are expressed in the glomerular podocytes. Both nephrin and Neph-family members function in cell adhesion and signalling, and thus regulate the structure and function of podocytes and maintain normal glomerular ultrafiltration. The expression of nephrin and Neph3 is altered in human proteinuric diseases emphasizing the importance of studying the transcriptional regulation of the nephrin and Neph3 genes NPHS1 (nephrosis 1, congenital, Finnish type) and KIRREL2 (kin of IRRE-like 2) respectively. The nephrin and Neph3 genes form a bidirectional gene pair, and they share transcriptional regulatory mechanisms. In the present review, we summarize the current knowledge of the functions of nephrin and Neph-family proteins and transcription factors and agents that control nephrin and Neph3 gene expression.
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Review Article|
October 25 2013
Functions of the podocyte proteins nephrin and Neph3 and the transcriptional regulation of their genes
Mervi Ristola;
Mervi Ristola
*Department of Pathology, Haartman Institute, University of Helsinki, 00290 Helsinki, Finland
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Sanna Lehtonen
*Department of Pathology, Haartman Institute, University of Helsinki, 00290 Helsinki, Finland
Correspondence: Dr Sanna Lehtonen (email sanna.h.lehtonen@helsinki.fi).
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Publisher: Portland Press Ltd
Received:
May 28 2013
Revision Received:
August 09 2013
Accepted:
August 21 2013
Online ISSN: 1470-8736
Print ISSN: 0143-5221
© The Authors Journal compilation © 2014 Biochemical Society
2014
Clin Sci (Lond) (2014) 126 (5): 315–328.
Article history
Received:
May 28 2013
Revision Received:
August 09 2013
Accepted:
August 21 2013
Citation
Mervi Ristola, Sanna Lehtonen; Functions of the podocyte proteins nephrin and Neph3 and the transcriptional regulation of their genes. Clin Sci (Lond) 1 March 2014; 126 (5): 315–328. doi: https://doi.org/10.1042/CS20130258
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