Finally, the role uPAR and suPAR may play in proteinuria as well as the binding and activation of plasminogen to plasmin on the surface of renal endothelial, podocyte, and tubular cells of patients with Fabry disease is another subject to be studied, as uPAR/suPAR could be involved in the pathogenesis of proteinuria and amiloride could be a potential adjunctive tool to reduce it, modulating inflammation and thrombogenic mechanisms in Fabry disease. Here, PLAUR is linked to Fabry disease.