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Journal of Lipid Research, Vol 28, 968-972, Copyright © 1987 by Lipid Research, Inc.
T Tokoro, AE Gal, LL Gallo and RO Brady
The time course of the clearance from the blood and the tissue localization
of [14C]L-glucosylceramide, a nonmetabolizable enantiomorph of
D-glucosylceramide that accumulates in Gaucher's disease, has been
determined. 14C-labeled L-glucosylceramide injected intravenously in the
form of micelles or liposomes is rapidly removed from the circulation. Most
of this lipid is taken up by the liver where it is found in both
hepatocytes and nonparenchymal cells. This sphingolipid analog is promptly
cleared from hepatocytes and a significant portion is recovered in the
bile. The clearance of [14C]L- glucosylceramide from Kupffer cells is
greatly prolonged in comparison with its brief residence in hepatocytes.
These findings have significant implications regarding the pathogenesis and
treatment of Gaucher's disease.
ARTICLES
Studies of the pathogenesis of Gaucher's disease: tissue distribution and biliary excretion of [14C]L-glucosylceramide in rats
Developmental and Metabolic Neurology Branch, National Institute of Neurological and Communicative Disorders and Stroke, Bethesda, MD 20892.
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