Transformation of 5α-cholest-7-en-3β-ol to cholesterol and cholestanol in cerebrotendinous xanthomatosis

The metabolism of Δ7-cholestenol, cholesterol, and cholestanol was examined in a patient with cerebrotendinous xanthomatosis after intravenous pulse-labeling with a mixture of dl-[2-14C]mevalonate and stereospecific 3S,4S,3R,4R-[4-3H]-mevalonate. Silver nitrate and reversed-phase thin-layer chromato...

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Main Authors: G.S. Tint, Gerald Salen
Format: Article
Language:English
Published: Elsevier 1974-05-01
Series:Journal of Lipid Research
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S0022227520368048
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author G.S. Tint
Gerald Salen
author_facet G.S. Tint
Gerald Salen
author_sort G.S. Tint
collection DOAJ
description The metabolism of Δ7-cholestenol, cholesterol, and cholestanol was examined in a patient with cerebrotendinous xanthomatosis after intravenous pulse-labeling with a mixture of dl-[2-14C]mevalonate and stereospecific 3S,4S,3R,4R-[4-3H]-mevalonate. Silver nitrate and reversed-phase thin-layer chromatography were used to purify the sterols isolated from the feces, and their identities were confirmed by gas–liquid chromatography–mass spectrometry. The specific activities were determined and plotted as a function of time. Isotope ratio measurements and specific activity decay curves showed that sterol synthesis proceeded in the following sequence: mevalonate, squalene, lanosterol, Δ7-cholestenol, cholesterol, cholestanol. Labeled cholesterol precursors might be advantageously used to measure changes in cholesterol synthesis because they appear to equilibrate rapidly and have very short turnover times.
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spelling doaj.art-7a1795041f6f49ccbd9529d4b9c672622022-12-21T23:18:13ZengElsevierJournal of Lipid Research0022-22751974-05-01153256262Transformation of 5α-cholest-7-en-3β-ol to cholesterol and cholestanol in cerebrotendinous xanthomatosisG.S. Tint0Gerald Salen1Gastroenterology Sections, Veterans Administration Hospitals, New York 10010; Gastroenterology Sections, Veterans Administration Hospitals, East Orange, New Jersey 07019, and; Department of Medicine, College of Medicine and Dentistry of New Jersey, Newark, New Jersey 07103Gastroenterology Sections, Veterans Administration Hospitals, New York 10010; Gastroenterology Sections, Veterans Administration Hospitals, East Orange, New Jersey 07019, and; Department of Medicine, College of Medicine and Dentistry of New Jersey, Newark, New Jersey 07103The metabolism of Δ7-cholestenol, cholesterol, and cholestanol was examined in a patient with cerebrotendinous xanthomatosis after intravenous pulse-labeling with a mixture of dl-[2-14C]mevalonate and stereospecific 3S,4S,3R,4R-[4-3H]-mevalonate. Silver nitrate and reversed-phase thin-layer chromatography were used to purify the sterols isolated from the feces, and their identities were confirmed by gas–liquid chromatography–mass spectrometry. The specific activities were determined and plotted as a function of time. Isotope ratio measurements and specific activity decay curves showed that sterol synthesis proceeded in the following sequence: mevalonate, squalene, lanosterol, Δ7-cholestenol, cholesterol, cholestanol. Labeled cholesterol precursors might be advantageously used to measure changes in cholesterol synthesis because they appear to equilibrate rapidly and have very short turnover times.http://www.sciencedirect.com/science/article/pii/S0022227520368048β-sitosterolgas–liquid chromatographymass spectrometrysilver nitrate thin-layer chromatographyreversed-phase thin-layer chromatographytwo-pool model
spellingShingle G.S. Tint
Gerald Salen
Transformation of 5α-cholest-7-en-3β-ol to cholesterol and cholestanol in cerebrotendinous xanthomatosis
Journal of Lipid Research
β-sitosterol
gas–liquid chromatography
mass spectrometry
silver nitrate thin-layer chromatography
reversed-phase thin-layer chromatography
two-pool model
title Transformation of 5α-cholest-7-en-3β-ol to cholesterol and cholestanol in cerebrotendinous xanthomatosis
title_full Transformation of 5α-cholest-7-en-3β-ol to cholesterol and cholestanol in cerebrotendinous xanthomatosis
title_fullStr Transformation of 5α-cholest-7-en-3β-ol to cholesterol and cholestanol in cerebrotendinous xanthomatosis
title_full_unstemmed Transformation of 5α-cholest-7-en-3β-ol to cholesterol and cholestanol in cerebrotendinous xanthomatosis
title_short Transformation of 5α-cholest-7-en-3β-ol to cholesterol and cholestanol in cerebrotendinous xanthomatosis
title_sort transformation of 5α cholest 7 en 3β ol to cholesterol and cholestanol in cerebrotendinous xanthomatosis
topic β-sitosterol
gas–liquid chromatography
mass spectrometry
silver nitrate thin-layer chromatography
reversed-phase thin-layer chromatography
two-pool model
url http://www.sciencedirect.com/science/article/pii/S0022227520368048
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