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Journal of Translational Medicine

, 14:289



BackgroundCertain 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.

MethodsCross-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.

ResultsControls 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.

ConclusionsFabry 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.

KeywordsEnzyme replacement therapy Fabry disease Lyso-Gb3 Podocyte Podocyturia CD80 Proteinuria AbbreviationsCD74cluster of differentiation 74

CD80 B7-1lymphocyte activation antigen 7-1

CKD-EPIchronic kidney disease-epidemiology collaboration

CTLA-4cytotoxic T-lymphocyte protein 4


eGFRestimated glomerular filtration rate

ERTenzyme replacement therapy

FGF-2fibroblast growth factor-2

GAPDHglyceraldehyde 3-phosphate dehydrogenase


GLAgalactosidase alpha

GSK 3glycogen synthase kinase

ITSinsulin, transferrin, selenite


MCP1monocyte chemoattractant protein-1

NFκBnuclear factor kappa B

PBSphosphate-buffered saline

PCRpolymerase chain reaction

RNAribonucleic acid

TGF-β1transforming growth factor-β1

UPCRurinary protein-creatinine ratio

VEGFvascular endothelial growth factor

Sanchez-Niño MD and Ortiz A are contributed equally to this work

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Autor: H. Trimarchi - R. Canzonieri - A. Schiel - C. Costales-Collaguazo - J. Politei - A. Stern - M. Paulero - T. Rengel - J. A


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