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Journal of Lipid Research, Vol 26, 1126-1133, Copyright © 1985 by Lipid Research, Inc.
G Salen, I Horak, M Rothkopf, JL Cohen, J Speck, GS Tint, V Shore, B Dayal, T Chen and S Shefer
Tissue sterol composition was determined in an 18-year-old male with
sitosterolemia with xanthomatosis who died suddenly and whose coronary and
aortic vessels showed extensive atherosclerosis and, for comparison, in an
18-year-old male with minimal atherosclerosis who died accidently. Sterols
in the control tissues (plasma, erythrocytes, cardiac muscle, lung, liver,
aorta, and brain) contained cholesterol with only trace amounts of
cholestanol. In contrast, sterols in corresponding tissues of the
sitosterolemic subject (except brain) were composed of cholesterol,
increased amounts of plant sterols, campesterol and sitosterol, and 5
alpha-saturated stanols, cholestanol, 5 alpha-campestanol, and 5
alpha-sitostanol, that were deposited in approximately the same ratio as
present in plasma. However, sitosterolemic brain sterol composition
resembled that of the control brain with cholesterol and only trace amounts
(less than 1%) of cholestanol and phytosterols. The sitosterolemic aorta
was extensively atherosclerotic and contained more than twice the quantity
of sterols as the control aorta (5.6 mg/g versus 2.6 mg/g) with increased
amounts of cholesterol, plant sterols, and 5 alpha-saturated stanols. These
results indicate that cholesterol, plant sterols, and 5 alpha-stanols are
deposited prematurely and are associated with accelerated atherosclerosis
in subjects with sitosterolemia with xanthomatosis.
ARTICLES
Lethal atherosclerosis associated with abnormal plasma and tissue sterol composition in sitosterolemia with xanthomatosis
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