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International Journal of Nephrology
Volume 2017, Article ID 1287289, 7 pages
Research Article

Expression of uPAR in Urinary Podocytes of Patients with Fabry Disease

1Nephrology Service, Hospital Británico de Buenos Aires, Buenos Aires, Argentina
2Central Laboratory, Hospital Británico de Buenos Aires, Buenos Aires, Argentina
3Neurology Department, Laboratorio de Neuroquímica Dr. Nestor Chamoles, Buenos Aires, Argentina
4IFIBIO Houssay, CONICET, Physiopathology, Pharmacy and Biochemistry Faculty, Universidad de Buenos Aires, Buenos Aires, Argentina
5IIS-Fundación Jimenez Diaz, School of Medicine, UAM, Madrid, Spain
6REDINREN, Madrid, Spain

Correspondence should be addressed to Hernán Trimarchi; moc.liamtoh@ihcramirth

Received 14 January 2017; Revised 11 March 2017; Accepted 19 March 2017; Published 24 April 2017

Academic Editor: Jochen Reiser

Copyright © 2017 Hernán Trimarchi et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Background. Despite enzyme replacement therapy, Fabry nephropathy still progresses. Podocyturia is an irreversible event that antedates proteinuria and leads to chronic renal failure. We evaluated a potential mechanism of podocyte detachment via the expression of the urokinase-type Plasminogen Activator Receptor (uPAR) in urinary podocytes of Fabry patients. Methods. This is a cross-sectional study that included controls () and Fabry patients () either untreated () or treated with agalsidase-β (). Variables. Variables are estimated glomerular filtration rate (eGFR), urinary protein : creatinine ratio, and urinary uPAR+ podocyte : creatinine ratio. uPAR mRNA expression in response to lyso-Gb3, a bioactive glycolipid accumulated in Fabry disease, was studied in cultured human podocytes. Results. Controls and Fabry patients had similar age, gender, and renal function. Urinary uPAR+ podocytes were higher in patients than in controls. Untreated patients were significantly younger; had more females, and presented lower urinary protein : creatinine ratios and significantly higher urinary uPAR+ podocytes than treated subjects. In treated patients, urinary uPAR+ podocytes correlated with urinary protein : creatinine ratio (; ). Lyso-Gb3 at concentrations found in the circulation of Fabry patients increased uPAR expression in cultured podocytes. Conclusions. Urinary podocytes expressing uPAR are increased in Fabry patients, especially in untreated patients. The potential contribution of uPAR expression to podocyte detachment merits further studies.