2007 JASN IMPACT FACTOR 7.111	HOME   AUTHOR INFO   EDITORIAL BOARD   SUBSCRIBE   FEEDBACK   ALERTS   HELP
advanced	CURRENT ISSUE	ARCHIVES	JASN Express	ONLINE SUBMISSION

This Article Full Text Full Text (PDF) All Versions of this Article: ASN.2005050509v1 16/11/3350    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Plaisier, E. Articles by Ronco, P. Search for Related Content PubMed PubMed Citation Articles by Plaisier, E. Articles by Ronco, P.

Glomerular Permeability Is Altered by Loss of P0, a Myelin Protein Expressed in Glomerular Epithelial Cells

Emmanuelle Plaisier^*, Béatrice Mougenot^*, Marie Christine Verpont^*, Chantal Jouanneau^*, Joan J. Archelos, Rudolf Martini, Dontscho Kerjaschki and Pierre Ronco^*

^* Department of Nephrology, INSERM Unit 702, Tenon Hospital (AP-HP), University Pierre et Marie Curie, Paris, France; Department of Neurology, University of Graz, Graz, Austria; Department of Developmental Neurobiology, University of Würzburg, Würzburg, Germany; and Department of Clinical Pathology, University of Vienna, Vienna, Austria

Address correspondence to: Dr. Emmanuelle Plaisier, Department of Nephrology and INSERM Unit 702, Hôpital Tenon, 4 Rue de la Chine, Paris, 75020 France. Phone: +33-1-56-01-66-39; Fax: +33-1-56-01-69-99; emmanuelle.plaisier{at}tnn.ap-hop-paris.fr

Received for publication May 16, 2005. Accepted for publication July 20, 2005.

The myelin protein 0 (MPZ or P0) is a transmembrane glycoprotein that represents the most abundant myelin component. Mutations in the P0 gene are associated with one form of autosomal dominant demyelinating peripheral neuropathy, Charcot-Marie-Tooth disease type 1B (CMT1B). Because CMT1 may be associated with renal involvement, mostly focal segmental glomerulosclerosis, we hypothesized that P0 could be expressed in the kidney. P0 mRNA was detected by reverse transcriptase–PCR in the human and mouse renal cortex. P0 transcripts were identified by in situ hybridization at different stages of the mouse kidney development, especially in embryonic structures that give rise to the glomerulus. P0 protein was also detected by Western blot in human and rat glomerular extracts and in a human podocyte cell line using a monoclonal anti-P0 antibody. Immunofluorescence studies on human kidney sections showed that the podocytes were intensely labeled. Immunogold electron microscopy disclosed a predominant staining of the membranes of intracellular vesicles in podocytes. P0 was also detected in the podocyte cell membrane, including at the foot processes. P0^–/– mice exhibited mild growth retardation and demyelinating neuropathy similar to the one observed in patients with CMT1B. They also presented mild albuminuria, without significant ultrastructural change of the glomerular basement membrane or the podocytes. These results demonstrate that P0, the major myelin protein, is also expressed during nephrogenesis and in mature kidney, mostly in podocytes. They suggest that P0 gene mutations might be involved in renal diseases.

This article has been cited by other articles:

				N. Miyauchi, A. Saito, T. Karasawa, Y. Harita, K. Suzuki, H. Koike, G. D. Han, F. Shimizu, and H. Kawachi Synaptic Vesicle Protein 2B Is Expressed in Podocyte, and Its Expression Is Altered in Proteinuric Glomeruli J. Am. Soc. Nephrol., October 1, 2006; 17(10): 2748 - 2759. [Abstract] [Full Text] [PDF]

Copyright © 2008 by the American Society of Nephrology. Online ISSN: 1533-3450 Print ISSN: 1046-6673