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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Format: | Article |
Language: | English |
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Elsevier
1974-05-01
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Series: | Journal of Lipid Research |
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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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id | doaj.art-7a1795041f6f49ccbd9529d4b9c67262 |
institution | Directory Open Access Journal |
issn | 0022-2275 |
language | English |
last_indexed | 2024-12-14T03:51:00Z |
publishDate | 1974-05-01 |
publisher | Elsevier |
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series | Journal of Lipid Research |
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 |
work_keys_str_mv | AT gstint transformationof5acholest7en3boltocholesterolandcholestanolincerebrotendinousxanthomatosis AT geraldsalen transformationof5acholest7en3boltocholesterolandcholestanolincerebrotendinousxanthomatosis |