Functions of the podocyte proteins nephrin and Neph3 and the transcriptional regulation of their genes.

Abstract

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.

• Authors: Mervi Ristola, Sanna Lehtonen
• Journal: Clinical science (London, England : 1979) (Clin Sci (Lond)) Vol. 126 Issue 5 Pg. 315-28 (Mar 2014) ISSN: 1470-8736 [Electronic] England
• PMID: 24219158 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Review)

Chemical References

• Immunoglobulins
• KIRREL2 protein, human
• Membrane Proteins
• nephrin

Topics

• DNA Methylation
• Gene Expression Regulation (physiology)
• Humans
• Immunoglobulins (chemistry, genetics, physiology)
• Membrane Proteins (chemistry, genetics, physiology)
• Podocytes (metabolism)
• Promoter Regions, Genetic
• Signal Transduction
• Structure-Activity Relationship
• Transcription, Genetic (physiology)