Lethal atherosclerosis associated with abnormal plasma and tissue sterol composition in sitosterolemia with xanthomatosis

G. Salen, I. Horak, M. Rothkopf, J. L. Cohen, J. Speck, G. S. Tint, V. Shore, B. Dayal, T. Chen, S. Shefer

Research output: Contribution to journalArticle

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Abstract

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 5α-saturated stanols, cholestanol, 5α-campestanol, and 5α-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 (< 1%) of cholestanol and phytosterols. The sitosterolemic aorta was extensively atherosclerotic and contained more than twice the quantity of serols as the control aorta (5.6 mg/g versus 2.6 mg/g) with increased amounts of cholesterol, plant serols, and 5α-saturated stanols. These results indicate that cholesterol, plant sterols, and 5α-sterols are deposited prematurely and are associated with accelerated atherosclerosis in subjects with sitosterolemia with xanthomatosis.

Original languageEnglish (US)
Pages (from-to)1126-1133
Number of pages8
JournalJournal of Lipid Research
Volume26
Issue number9
StatePublished - Dec 1 1985
Externally publishedYes

Fingerprint

Xanthomatosis
Sterols
Cholestanol
Atherosclerosis
Phytosterols
Cholesterol
Tissue
Brain
Plasmas
Aorta
Chemical analysis
Liver
Muscle
Coronary Vessels
Myocardium
Erythrocytes
Sitosterolemia
Lung

All Science Journal Classification (ASJC) codes

  • Biochemistry
  • Endocrinology
  • Cell Biology

Cite this

Salen, G. ; Horak, I. ; Rothkopf, M. ; Cohen, J. L. ; Speck, J. ; Tint, G. S. ; Shore, V. ; Dayal, B. ; Chen, T. ; Shefer, S. / Lethal atherosclerosis associated with abnormal plasma and tissue sterol composition in sitosterolemia with xanthomatosis. In: Journal of Lipid Research. 1985 ; Vol. 26, No. 9. pp. 1126-1133.
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abstract = "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 5α-saturated stanols, cholestanol, 5α-campestanol, and 5α-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 (< 1{\%}) of cholestanol and phytosterols. The sitosterolemic aorta was extensively atherosclerotic and contained more than twice the quantity of serols as the control aorta (5.6 mg/g versus 2.6 mg/g) with increased amounts of cholesterol, plant serols, and 5α-saturated stanols. These results indicate that cholesterol, plant sterols, and 5α-sterols are deposited prematurely and are associated with accelerated atherosclerosis in subjects with sitosterolemia with xanthomatosis.",
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Salen, G, Horak, I, Rothkopf, M, Cohen, JL, Speck, J, Tint, GS, Shore, V, Dayal, B, Chen, T & Shefer, S 1985, 'Lethal atherosclerosis associated with abnormal plasma and tissue sterol composition in sitosterolemia with xanthomatosis', Journal of Lipid Research, vol. 26, no. 9, pp. 1126-1133.

Lethal atherosclerosis associated with abnormal plasma and tissue sterol composition in sitosterolemia with xanthomatosis. / Salen, G.; Horak, I.; Rothkopf, M.; Cohen, J. L.; Speck, J.; Tint, G. S.; Shore, V.; Dayal, B.; Chen, T.; Shefer, S.

In: Journal of Lipid Research, Vol. 26, No. 9, 01.12.1985, p. 1126-1133.

Research output: Contribution to journalArticle

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T1 - Lethal atherosclerosis associated with abnormal plasma and tissue sterol composition in sitosterolemia with xanthomatosis

AU - Salen, G.

AU - Horak, I.

AU - Rothkopf, M.

AU - Cohen, J. L.

AU - Speck, J.

AU - Tint, G. S.

AU - Shore, V.

AU - Dayal, B.

AU - Chen, T.

AU - Shefer, S.

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N2 - 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 5α-saturated stanols, cholestanol, 5α-campestanol, and 5α-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 (< 1%) of cholestanol and phytosterols. The sitosterolemic aorta was extensively atherosclerotic and contained more than twice the quantity of serols as the control aorta (5.6 mg/g versus 2.6 mg/g) with increased amounts of cholesterol, plant serols, and 5α-saturated stanols. These results indicate that cholesterol, plant sterols, and 5α-sterols are deposited prematurely and are associated with accelerated atherosclerosis in subjects with sitosterolemia with xanthomatosis.

AB - 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 5α-saturated stanols, cholestanol, 5α-campestanol, and 5α-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 (< 1%) of cholestanol and phytosterols. The sitosterolemic aorta was extensively atherosclerotic and contained more than twice the quantity of serols as the control aorta (5.6 mg/g versus 2.6 mg/g) with increased amounts of cholesterol, plant serols, and 5α-saturated stanols. These results indicate that cholesterol, plant sterols, and 5α-sterols are deposited prematurely and are associated with accelerated atherosclerosis in subjects with sitosterolemia with xanthomatosis.

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