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Abstract
To the Editor: In their review, Quehenberger and Dennis (Nov. 10 issue)1 describe plasma lipids implicated in Gaucher's disease. Although they could not possibly mention every lipid, we believe it is worth commenting on the cationic amphiphilic glycolipid globotriaosylsphingosine (lyso-Gb3) and its contribution to a better understanding of the pathogenesis and monitoring of Fabry's disease. High plasma concentrations of lyso-Gb3 were observed in patients with this disease,2 and these levels correlated with several of its manifestations3 and decreased in response to enzyme-replacement therapy.4,5 Furthermore, lyso-Gb3 promoted vascular smooth-muscle cell proliferation2 as well as transforming growth factor-β1–mediated synthesis of extracellular . . .
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1 Instituto de Investigación Sanitaria de la Fundación Jiménez Díaz, Madrid, Spain