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Journal of Lipid Research, Vol 33, 945-955, Copyright © 1992 by Lipid Research, Inc.
G Salen, S Shefer, L Nguyen, GC Ness, GS Tint and V Shore
Sitosterolemia is a rare inherited lipid storage disease characterized
chemically by the accumulation of plant sterols and 5 alpha-saturated
stanols in plasma and tissues. Very low cholesterol synthesis due to a
deficiency of HMG-CoA reductase associated with increased intestinal plant
sterol absorption and slow hepatic sterol removal are major biochemical
features. Because cholesterol synthesis cannot up-regulate, bile acid
malabsorption mobilizes body sterols for bile acid synthesis and
dramatically lowers plasma and monocyte sterol concentrations and may halt
the progression of the atherosclerotic process.
REVIEWS
Sitosterolemia
Department of Medicine, UMD-New Jersey Medical School, Newark 07103.
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