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

G. Salen, P. O. Kwiterovich, S. Shefer, G. S. Tint, I. Horak, V. Shore, B. Dayal, E. Horak

Research output: Contribution to journalArticlepeer-review

Abstract

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 5α-saturated stanols, cholestanol, 5α-campestanol, and 5α-sitostanol were at least 10 times greater than controls. Diets contained plentiful quantities of cholesterol and plant sterols, but only trace amounts of cholestanol (<2 mg/day) and no detectable 5α-campestanol and 5α-sitostanol, which indicated that the 5α-saturated stanols were formed endogenously. Treatment with cholestyramine reduced plasma cholesterol and phytosterol levels by 45% and 5α-saturated stanols by 55%. These results indicate that abnormally high plasma concentrations of cholestanol, 5α-campestanol, and 5α-sitostanol are found in subjects with sitosterolemia and xanthomatosis, and that treatment with cholestyramine effectively reduced elevated plasma sterol levels.

Original languageEnglish (US)
Pages (from-to)203-209
Number of pages7
JournalJournal of Lipid Research
Volume26
Issue number2
StatePublished - 1985

ASJC Scopus subject areas

  • Biochemistry
  • Endocrinology
  • Cell Biology

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