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Increased urinary CD80 excretion and podocyturia in Fabry disease
H Trimarchi1, R Canzonieri2, A Schiel2
1Nephrology Service, Hospital Británico de Buenos Aires, Perdriel 74, 1280, Buenos Aires, Argentina. htrimarchi@hotmail.com.
Journal of Translational Medicine
|October 14, 2016
Summary
Urinary CD80 (uCD80) excretion is elevated in Fabry disease patients, correlating with proteinuria. This suggests uCD80 may indicate early kidney damage from glycolipid accumulation in Fabry nephropathy.
Area of Science:
- Nephrology
- Biochemistry
- Genetics
Background:
- Certain glomerulopathies are linked to elevated CD80 (B7-1) levels.
- Fabry disease is a genetic disorder causing glycolipid accumulation and kidney damage.
Purpose of the Study:
- To measure urinary CD80 (uCD80) excretion, podocyturia, and proteinuria in controls and Fabry disease patients.
- To investigate the relationship between uCD80 and kidney damage markers in Fabry disease.
- To explore the effect of lyso-Gb3 on CD80 expression in podocytes.
Main Methods:
- A cross-sectional study involving 65 individuals (20 controls, 45 Fabry patients on or off enzyme replacement therapy (ERT)).
- Measured urinary protein/creatinine ratio (UPCR), estimated glomerular filtration rate (eGFR), urinary uCD80/creatinine ratio (uCD80), and podocyturia.
- Studied CD80 mRNA expression in cultured human podocytes exposed to lyso-Gb3.
Main Results:
- Fabry patients exhibited significantly higher UPCR, uCD80, and podocyturia compared to controls.
- uCD80 and UPCR showed a significant positive correlation in the overall cohort and in Fabry patients.
- Lyso-Gb3 increased uCD80 expression in cultured podocytes.
Conclusions:
- Fabry disease is associated with early increases in uCD80 excretion, likely due to glycolipid accumulation.
- uCD80 may serve as an early biomarker for subclinical renal involvement in Fabry disease.
- Further research is warranted to explore the diagnostic potential of uCD80 in Fabry nephropathy.
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