Sphingosine-1 phosphate analogues as cardiac protectors in Fabry disease

17 Mar 2010 | News

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Researchers at University and University Hospital Lausanne have discovered that Sphingosine-1 phosphate (S1P) analogues act as cardiac protectors in the inherited disorder Fabry disease. They believe the compounds could also be used as the basis of treatment for cardiac hypertrophy due to other causes.

Fabry disease is an inherited metabolic disorder in which many patients have left ventricular hypertrophy and increased thickness of the arteries, leading to shorter life expectancy. These complications are clinically indistinguishable from similar abnormalities that occur in more common cardiac disorders in the general population.

The researchers have shown that:

  • plasma from Fabry patients triggers high metabolic activity in vascular smooth muscle cells;

  • the concentration of S1P is higher in plasma from Fabry patients;

  • S1P has a direct effect on both vascular smooth muscle cell hypertrophy and proliferation. In contrast S1P antagonists block the hypertrophic effect of S1P;

  • proof of concept has been demonstrated in vitro and in mouse models

Additional information is available on request.

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