In:
International Journal of Nephrology, Hindawi Limited, Vol. 2017 ( 2017), p. 1-7
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 : 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.
Type of Medium:
Online Resource
ISSN:
2090-214X
,
2090-2158
DOI:
10.1155/2017/1287289
Language:
English
Publisher:
Hindawi Limited
Publication Date:
2017
detail.hit.zdb_id:
2573904-9
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