Journal
JOURNAL OF TRANSLATIONAL MEDICINE
Volume 14, Issue -, Pages -Publisher
BIOMED CENTRAL LTD
DOI: 10.1186/s12967-016-1049-8
Keywords
Enzyme replacement therapy; Fabry disease; Lyso-Gb3; Podocyte; Podocyturia; CD80; Proteinuria
Categories
Funding
- FIS [PI13/00047, CP14/00133, PI15/00298, PI16/02057]
- FEDER funds ISCIII-RETIC REDinREN [RD12/0021]
- IBERERC
- Sociedad Espanola de Nefrologia
- Programa Intensificacion Actividad Investigadora (ISCIII/Agencia Lain-Entralgo/CM)
- Miguel Servet [MS14/00133]
- VI Convocatoria de ayudas investigacion sobre medicamentos huerfanos y enfermedades raras con la ayuda Fundacion Cajasol
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Background: Certain glomerulopathies are associated with increased levels of CD80 (B7-1). We measured the urinary excretion of CD80, podocyturia and proteinuria in controls and in subjects with Fabry disease either untreated or on enzyme replacement therapy (ERT). Methods: Cross-sectional study including 65 individuals: controls (n = 20) and Fabry patients (n = 45, 23 of them not on ERT and 22 on ERT). Variables included age, gender, urinary protein/creatinine ratio (UPCR), estimated glomerular filtration rate (eGFR), urinary uCD80/creatinine ratio (uCD80) and podocyturia. CD80 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 did not differ in age, eGFR and gender. However, UPCR, uCD80 and podocyturia were significantly higher in Fabry patients than in controls. As expected, Fabry patients not on ERT were younger and a higher percentage were females. Non-ERT Fabry patients had less advanced kidney disease than ERT Fabry patients: UPCR was lower and eGFR higher, but uCD80 and podocyturia did not differ between non-ERT or ERT Fabry patients. There was a significant correlation between uCD80 and UPCR in the whole population (r 0.44, p 0.0005) and in Fabry patients (r 0.42, p 0.0046). Lyso-Gb3 at concentrations found in the circulation of Fabry patients increased uCD80 expression in cultured podocytes. Conclusions: Fabry disease is characterized by early occurrence of increased uCD80 excretion that appears to be a consequence of glycolipid accumulation. The potential for uCD80 excretion to reflect early, subclinical renal Fabry involvement should be further studied.
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