Early decrease in the podocalyxin to synaptopodin ratio in urinary Fabry podocytes
| Autor: | Tatiana Rengel, Romina Canzonieri, Mariano Forrester, Romina Iriarte, Vanesa Pomeranz, Cristian Costales-Collaguazo, Aníbal Stern, Alexis Muryan, Elsa Zotta, Juan Politei, Fernando Lombi, Matías Paulero, Hernán Trimarchi, Amalia Schiel, Ivan Gonzalez-Hoyos |
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| Rok vydání: | 2018 |
| Předmět: |
medicine.medical_specialty
podocyte 030232 urology & nephrology Urology Renal function Familial Nephropathies Nephropathy Podocyte Fabry nephropathy 03 medical and health sciences chemistry.chemical_compound synaptopodin 0302 clinical medicine podocyturia medicine Transplantation Proteinuria business.industry Glomerulosclerosis Enzyme replacement therapy podocalyxin medicine.disease medicine.anatomical_structure Fabry Podocalyxin chemistry Nephrology Synaptopodin medicine.symptom business |
| Zdroj: | Clinical Kidney Journal |
| ISSN: | 2048-8513 2048-8505 |
| Popis: | Background In Fabry nephropathy, podocyturia is an early event that may lead to glomerulosclerosis and chronic kidney disease. The glycocalyx is a potential podocyte damaged compartment in glomerulopathies. We investigated glycocalyx podocalyxin in urinary detached podocytes compared with cytoplasmic synaptopodin. Methods This was a cross-sectional study including 68 individuals: Controls (n = 20) and Fabry patients (n = 48), 15 untreated and 33 treated. Variables included age, gender, urinary protein/creatinine ratio (UPCR), estimated glomerular filtration rate (eGFR), lyso-triasocylsphingosine (lyso-Gb3) levels and enzyme replacement therapy (ERT). Podocyturia was assessed by immunofluorescence and podocyte subpopulations were analyzed. Results Fabry patients displayed higher podocyturia than controls. Fabry treated subjects (n = 33) presented significantly higher UPCR compared with untreated ones (n = 15); podocyturia, eGFR and lyso-Gb3 levels were not different. All control podocytes colocalized synaptopodin and podocalyxin; 13 Fabry patients (27%) colocalized these proteins, while 35 (73%) were only synaptopodin positive. No podocalyxin-positive/synaptopodin-negative cells were encountered. In Fabry patients, podocyturia was significantly higher and proteinuria lower in those that colocalized. Conclusion Fabry patients present higher podocyturia and a presumably more damaged glycocalyx assessed by podocalyxin. Treated patients had significant higher proteinuria suggesting ERT is initiated late, at advanced stages. The degree of podocalyxin-negative podocytes was similar in both groups, but colocalization was associated with lower proteinuria. Podocyturia assessed by podocalyxin alone may be underestimated. The implications of podocyte glycocalyx damage deserve further investigations. |
| Databáze: | OpenAIRE |
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