Increased plasma cholestanol and 5 alpha-saturated plant sterol derivatives in subjects with sitosterolemia and xanthomatosis.

• Main Authors: G Salen, P O Kwiterovich, Jr, S Shefer, G S Tint, I Horak, V Shore, B Dayal, E Horak
• Format: Article
• Language: English
• Published: Elsevier 1985-02-01
• Series: Journal of Lipid Research
• Online Access: http://www.sciencedirect.com/science/article/pii/S002222752034390X

We have measured plasma sterol composition in 14 subjects with sitosterolemia and xanthomatosis. In addition to elevated plasma phytosterol (campesterol 16 +/- 7 mg/dl and sitosterol 35 +/- 16 mg/dl) and normal to moderately high cholesterol levels (258 +/- 96 mg/dl), concentrations of 5 alpha-saturated stanols, cholestanol, 5 alpha-campestanol, and 5 alpha-sitostanol were at least 10 times greater than controls. Diets contained plentiful quantities of cholesterol and plant sterols, but only trace amounts of cholestanol (less than 2 mg/day) and no detectable 5 alpha-campestanol and 5 alpha-sitostanol, which indicated that the 5 alpha-saturated stanols were formed endogenously. Treatment with cholestyramine reduced plasma cholesterol and phytosterol levels by 45% and 5 alpha-saturated stanols by 55%. These results indicate that abnormally high plasma concentrations of cholestanol, 5 alpha-campestanol, and 5 alpha-sitostanol are found in subjects with sitosterolemia and xanthomatosis, and that treatment with cholestyramine effectively reduced elevated plasma sterol levels.