Journal of Lipid Research, Vol 32, 223-229, Copyright © 1991 by Lipid Research, Inc.
High levels of plant sterols and cholesterol precursors in cerebrotendinous xanthomatosis
M Kuriyama, J Fujiyama, T Kasama and M Osame
Third Department of Internal Medicine, Kagoshima University School of Medicine, Japan.
We measured the cholestanol, cholesterol precursor (lathosterol), and plant
sterol (campesterol and sitosterol) concentrations of serum and bile in 11
patients with cerebrotendinous xanthomatosis. The mean values of serum
cholestanol, lathosterol, campesterol, and sitosterol were, respectively,
8.4-, 2.5-, 2.7-, and 1.4-times higher in the patients than in normal
control subjects (n = 26). Cholestanol (6.7- fold) and campesterol
(3.7-fold) levels in bile (n = 4) were also elevated in the patients. There
was no significant difference of serum sterol levels between patients with
coronary artery disease and those without it. Chenodeoxycholic acid
treatment for periods ranging from 6 months to 3 years and 4 months lowered
serum lathosterol (57.7% reduction) and campesterol (57.8%) levels in
parallel with cholestanol (70.8%) level, but the sitosterol level (19.7%)
decreased less. Thus, increased levels of cholesterol precursor
(lathosterol), plant sterols (campesterol and sitosterol), and cholestanol
were found in the serum and bile in cerebrotendinous xanthomatosis.
Chenodeoxycholic acid treatment effectively reduced the levels of these
sterols, except for sitosterol.
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