Table of Contents Author Guidelines Submit a Manuscript
International Journal of Nephrology
Volume 2017, Article ID 1287289, 7 pages
https://doi.org/10.1155/2017/1287289
Research Article

Expression of uPAR in Urinary Podocytes of Patients with Fabry Disease

1Nephrology Service, Hospital Británico de Buenos Aires, Buenos Aires, Argentina
2Central Laboratory, Hospital Británico de Buenos Aires, Buenos Aires, Argentina
3Neurology Department, Laboratorio de Neuroquímica Dr. Nestor Chamoles, Buenos Aires, Argentina
4IFIBIO Houssay, CONICET, Physiopathology, Pharmacy and Biochemistry Faculty, Universidad de Buenos Aires, Buenos Aires, Argentina
5IIS-Fundación Jimenez Diaz, School of Medicine, UAM, Madrid, Spain
6REDINREN, Madrid, Spain

Correspondence should be addressed to Hernán Trimarchi; moc.liamtoh@ihcramirth

Received 14 January 2017; Revised 11 March 2017; Accepted 19 March 2017; Published 24 April 2017

Academic Editor: Jochen Reiser

Copyright © 2017 Hernán Trimarchi et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Linked References

  1. R. O. Brady, A. E. Gal, R. M. Bradley, E. Martensson, A. L. Warshaw, and L. Laster, “Enzymatic defect in Fabry's disease. Ceramidetrihexosidase deficiency,” New England Journal of Medicine, vol. 276, no. 21, pp. 1163–1167, 1967. View at Publisher · View at Google Scholar · View at Scopus
  2. A. Kanda, S. Nakao, S. Tsuyama, F. Murata, and T. Kanzaki, “Fabry disease: ultrastructural lectin histochemical analyses of lysosomal deposits,” Virchows Archiv, vol. 436, no. 1, pp. 36–42, 2000. View at Publisher · View at Google Scholar · View at Scopus
  3. H. Askari, C. R. Kaneski, C. Semino-Mora et al., “Cellular and tissue localization of globotriaosylceramide in Fabry disease,” Virchows Archiv, vol. 451, no. 4, pp. 823–834, 2007. View at Publisher · View at Google Scholar · View at Scopus
  4. R. J. Desnick and C. M. Eng, “α-Galactosidase A deficiency: Fabry disease,” in The Metabolic and Molecular Bases of Inherited Disease, McGraw-Hill, 2001. View at Google Scholar
  5. E. Lloyd-Evans, D. Pelled, C. Riebeling et al., “Glucosylceramide and glucosylsphingosine modulate calcium mobilization from brain microsomes via different mechanisms,” Journal of Biological Chemistry, vol. 278, no. 26, pp. 23594–23599, 2003. View at Publisher · View at Google Scholar · View at Scopus
  6. D. Pelled, S. Trajkovic-Bodennec, E. Lloyd-Evans, E. Sidransky, R. Schiffmann, and A. H. Futerman, “Enhanced calcium release in the acute neuronopathic form of Gaucher disease,” Neurobiology of Disease, vol. 18, no. 1, pp. 83–88, 2005. View at Publisher · View at Google Scholar · View at Scopus
  7. D. P. Germain, S. Waldek, M. Banikazemi et al., “Sustained, long-term renal stabilization after 54 months of agalsidase β therapy in patients with Fabry disease,” Journal of the American Society of Nephrology, vol. 18, no. 5, pp. 1547–1557, 2007. View at Publisher · View at Google Scholar · View at Scopus
  8. C. Tøndel, L. Bostad, K. K. Larsen et al., “Agalsidase benefits renal histology in young patients with Fabry disease,” Journal of the American Society of Nephrology, vol. 24, no. 1, pp. 137–148, 2013. View at Publisher · View at Google Scholar · View at Scopus
  9. H. Trimarchi, “Podocyturia. What is in a name?” Journal of Translational Internal Medicine, vol. 3, pp. 51–56, 2015. View at Google Scholar
  10. A. Ortiz, B. Cianciaruso, M. Cizmarik et al., “End-stage renal disease in patients with Fabry disease: natural history data from the Fabry Registry,” Nephrology Dialysis Transplantation, vol. 25, no. 3, pp. 769–775, 2010. View at Publisher · View at Google Scholar · View at Scopus
  11. H. Trimarchi, R. Canzonieri, A. Schiel et al., “Podocyturia is significantly elevated in untreated vs treated Fabry adult patients,” Journal of Nephrology, vol. 29, no. 6, pp. 791–797, 2016. View at Google Scholar
  12. M. Nagata, “Podocyte injury and its consequences,” Kidney International, vol. 89, no. 6, pp. 1221–1230, 2016. View at Publisher · View at Google Scholar · View at Scopus
  13. H. A. Chapman and Y. Wei, “Protease crosstalk with integrins: the urokinase receptor paradigm,” Thrombosis and Haemostasis, vol. 86, no. 1, pp. 124–129, 2001. View at Google Scholar
  14. C. Wei, C. C. Möller, M. M. Altintas et al., “Modification of kidney barrier function by the urokinase receptor,” Nature Medicine, vol. 14, no. 1, pp. 55–63, 2008. View at Publisher · View at Google Scholar
  15. H. Trimarchi, R. Canzonieri, A. Schiel et al., “Increased urinary CD80 excretion and podocyturia in Fabry disease,” Journal of Translational Medicine, vol. 14, article 289, 2016. View at Publisher · View at Google Scholar
  16. M. D. Sanchez-Niño, D. Carpio, A. B. Sanz, M. Ruiz-Ortega, S. Mezzano, and A. Ortiz, “Lyso-Gb3 activates Notch1 in human podocytes,” Human Molecular Genetics, vol. 24, no. 20, pp. 5720–5732, 2015. View at Publisher · View at Google Scholar · View at Scopus
  17. M. D. Sanchez-Niño, A. B. Sanz, S. Carrasco et al., “Globotriaosylsphingosine actions on human glomerular podocytes: implications for Fabry nephropathy,” Nephrology Dialysis Transplantation, vol. 26, no. 6, pp. 1797–1802, 2011. View at Publisher · View at Google Scholar · View at Scopus
  18. A. Ortiz, H. Husi, L. Gonzalez-Lafuente et al., “Mitogen-activated protein kinase 14 promotes AKI,” Journal of the American Society of Nephrology, vol. 28, no. 3, pp. 823–836, 2017. View at Publisher · View at Google Scholar
  19. E. Ruoslahti, “Integrins,” The Journal of Clinical Investigation, vol. 87, no. 1, pp. 1–5, 1991. View at Publisher · View at Google Scholar
  20. S. Adler, “Integrin matrix receptors in renal injury,” Kidney International Supplements, vol. 45, pp. S86–S89, 1994. View at Google Scholar
  21. K. Utsumi, K. Itoh, R. Kase et al., “Urinary excretion of the vitronectin receptor (integrin alpha V beta 3) in patients with Fabry disease,” Clinica Chimica Acta, vol. 279, no. 1-2, pp. 55–68, 1999. View at Google Scholar
  22. A. Fornoni, S. Merscher, and J. B. Kopp, “Lipid biology of the podocyte-new perspectives offer new opportunities,” Nature Reviews Nephrology, vol. 10, no. 7, pp. 379–388, 2014. View at Publisher · View at Google Scholar · View at Scopus
  23. K. T. Preissner, S. M. Kanse, and A. E. May, “Urokinase receptor: a molecular organizer in cellular communication,” Current Opinion in Cell Biology, vol. 12, no. 5, pp. 621–628, 2000. View at Google Scholar
  24. E. J. Brown, “Integrin-associated proteins,” Current Opinion in Cell Biology, vol. 14, no. 5, pp. 603–607, 2002. View at Google Scholar
  25. N. Sachs and A. Sonnenberg, “Cell-matrix adhesion of podocytes in physiology and disease,” Nature Reviews Nephrology, vol. 9, no. 4, pp. 200–210, 2013. View at Publisher · View at Google Scholar · View at Scopus
  26. B. Zhang, S. Xie, W. Shi, and Y. Yang, “Amiloride off-target effect inhibits podocyte urokinase receptor expression and reduces proteinuria,” Nephrology Dialysis Transplantation, vol. 27, no. 5, pp. 1746–1755, 2012. View at Publisher · View at Google Scholar
  27. H. Trimarchi, M. Forrester, F. Lombi et al., “Amiloride as an alternate adjuvant antiproteinuric agent in Fabry disease: the potential roles of plasmin and uPAR,” Case Reports in Nephrology, vol. 2014, Article ID 854521, 6 pages, 2014. View at Publisher · View at Google Scholar
  28. H. C. Liao, Y. H. Huang, Y. J. Chen et al., “Plasma globotriaosylsphingosine (lysoGb3) could be a biomarker for Fabry disease with a Chinese hotspot late-onset mutation (IVS4+919G>A). Clin Chim Acta,” in doi, pp. 426–114, 426, 114-120, 2013. View at Google Scholar
  29. J. M. Aerts, J. E. Groener, S. Kuiper et al., “Elevated globotriaosylsphingosine is a hallmark of Fabry disease,” Proceedings of the National Academy of Sciences of the United States of America, vol. 105, no. 8, pp. 2812–2817, 2008. View at Publisher · View at Google Scholar
  30. B. E. Smid, L. Van Der Tol, F. Cecchi et al., “Uncertain diagnosis of Fabry disease: consensus recommendation on diagnosis in adults with left ventricular hypertrophy and genetic variants of unknown significance,” International Journal of Cardiology, vol. 177, no. 2, pp. 400–408, 2014. View at Publisher · View at Google Scholar · View at Scopus
  31. M. Niemann, A. Rolfs, S. Störk et al., “Gene mutations versus clinically relevant phenotypes: lyso-gb3 defines Fabry disease,” Circulation: Cardiovascular Genetics, vol. 7, no. 1, pp. 8–16, 2014. View at Publisher · View at Google Scholar · View at Scopus
  32. O. Goker-Alpan, M. J. Gambello, G. H. Maegawa et al., “Reduction of plasma globotriaosylsphingosine levels after switching from agalsidase alfa to agalsidase beta as enzyme replacement therapy for Fabry disease,” JIMD Reports, vol. 25, pp. 95–106, 2016. View at Google Scholar
  33. K. H. Chen, Y. Chien, K. L. Wang et al., “Evaluation of pro-inflammatory prognostic biomarkers for fabry cardiomyopathy with enzyme replacement therapy,” Canadian Journal of Cardiology, vol. 32, pp. 1221.e1–1221.e9, 2016. View at Publisher · View at Google Scholar