TY - JOUR
A2 - Reiser, Jochen
AU - Trimarchi, Hernán
AU - Canzonieri, Romina
AU - Schiel, Amalia
AU - Politei, Juan
AU - Costales-Collaguazo, Cristian
AU - Stern, Aníbal
AU - Paulero, Matías
AU - Rengel, Tatiana
AU - Valiño-Rivas, Lara
AU - Forrester, Mariano
AU - Lombi, Fernando
AU - Pomeranz, Vanesa
AU - Iriarte, Romina
AU - Muryan, Alexis
AU - Ortiz, Alberto
AU - Sanchez-Niño, María Dolores
AU - Zotta, Elsa
PY - 2017
DA - 2017/04/24
TI - Expression of uPAR in Urinary Podocytes of Patients with Fabry Disease
SP - 1287289
VL - 2017
AB - 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 (n=20) and Fabry patients (n=44) either untreated (n=23) or treated with agalsidase-β (n=21). 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 (ρ=0.5; p=0.02). 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.
SN - 2090-214X
UR - https://doi.org/10.1155/2017/1287289
DO - 10.1155/2017/1287289
JF - International Journal of Nephrology
PB - Hindawi
KW -
ER -