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European Heart Journal Advance Access published online on September 22, 2009
European Heart Journal, doi:10.1093/eurheartj/ehp387
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Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2009. For permissions please email: journals.permissions@oxfordjournals.org

Evidence for a role of sphingosine-1 phosphate in cardiovascular remodelling in Fabry disease

Noureddine Brakch1,*, Olivier Dormond2, Soumeya Bekri3, Dela Golshayan4, Magali Correvon1, Lucia Mazzolai1, Beat Steinmann5 and Frédéric Barbey4,*

1 Service of Angiology and Vascular Medicine, Centre Hospitalier Universitaire Vaudois (CHUV) and University of Lausanne, 5 Rue Pierre Decker, 1011 Lausanne, Switzerland
2 Department of Visceral Surgery, CHUV and University of Lausanne, Lausanne, Switzerland
3 Department of Biochemistry, Hôpital Charles Nicolle, Centre Hospitalier Universitaire de Rouen, Rouen, France
4 Transplantation Center, CHUV and University of Lausanne, Lausanne, Switzerland
5 Division of Metabolism and Molecular Pediatrics, University Children's Hospital, Zürich, Switzerland

Received 27 April 2009; revised 30 July 2009; accepted 26 August 2009 * Corresponding author. Tel: +41 21 314 07 73, Fax: +41 21 314 07 61, Email: noureddine.brakch{at}chuv.ch (N.B.); Tel: +41 21 314 76 06, Fax: +41 21 314 11 75, Email: frederic.barbey{at}chuv.ch (F.B.)

Aims: A hallmark of Fabry disease is the concomitant development of left-ventricular hypertrophy and arterial intima–media thickening, the pathogenesis of which is thought to be related to the presence of a plasmatic circulating growth-promoting factor. We therefore characterized the plasma of patients with Fabry disease in order to identify this factor.

Methods and results: Using a classical biochemical strategy, we isolated and identified sphingosine-1 phosphate (S1P) as a proliferative factor present in the plasma of patients with Fabry disease. Plasma S1P levels were significantly higher in 17 patients with Fabry disease compared with 17 healthy controls (225 ± 40 vs. 164 ± 17 ng/mL; P = 0.005). There was a positive correlation between plasma S1P levels and both common carotid artery intima–media thickness and left-ventricular mass index (r2 = 0.47; P = 0.006 and r2 = 0.53; P = 0.0007, respectively). In an experimental model, mice treated with S1P developed cardiovascular remodelling similar to that observed in patients with Fabry disease.

Conclusion: Sphingosine-1 phosphate participates in cardiovascular remodelling in Fabry disease. Our findings have implications for the treatment of cardiovascular involvement in Fabry disease.

Key Words: Fabry disease • Left ventricular hypertrophy • Remodelling • Sphingosine-1 phosphate • Intima–media thickness


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