Expression of uPAR in Urinary Podocytes of Patients with Fabry Disease

International Journal of Nephrology
H TrimarchiElsa Zotta

Abstract

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 : creatin...Continue Reading

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Citations

Feb 13, 2019·Clinical Kidney Journal·Hernán TrimarchiElsa Zotta
Nov 19, 2020·Journal of Clinical Medicine·Hernán TrimarchiMaria Dolores Sánchez-Niño
May 1, 2021·Journal of Clinical Medicine·Clara Carnicer-CáceresGuillem Pintos-Morell

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Methods Mentioned

BETA
enzyme replacement therapy
biopsy
FCS
PCR

Software Mentioned

InfoStat

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