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fabry disease/triglyceride
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Non-invasive determination of myocardial lipid content in Fabry disease by 1H-MR spectroscopy.
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OBJECTIVE
In Fabry disease (FD), a progressive deposition of sphingolipids is reported in different organs. The present study applied 1H magnetic resonance spectroscopy (MRS) to investigate the myocardial lipid content in FD.
METHODS
In patients (PTS, n = 15) with genetically proven FD, 1H MRS of
Non invasive evaluation of endothelial function in patients with Anderson-Fabry disease.
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OBJECTIVE
Fabry's disease is an X-linked recessive abnormality of glycosphingolipid metabolism. Increased levels of endothelial prothrombotic factors have recently been demonstrated in Fabry's disease, whereas endothelial function has not been studied using high resolution ultrasound.
METHODS
We
Reduction of plasma glycosphingolipid levels has no impact on atherosclerosis in apolipoprotein E-null mice.
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Glycosphingolipids (GSLs) have been implicated as potential atherogenic lipids. Studies in apolipoprotein E-null (apoE(-/-)) mice indicate that exacerbated tissue GSL accumulation resulting from alpha-galactosidase deficiency promotes atherosclerosis, whereas the serine palmitoyl transferase
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