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Journal of Lipid Research, Vol. 15, 256-262, May 1974
Copyright © 1974 by Lipid Research, Inc.

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

G. S. Tint and Gerald Salen

Gastroenterology Sections, Veterans Administration Hospitals, New York 10010 and East Orange, New Jersey 07019, and Department of Medicine, College of Medicine and Dentistry of New Jersey, Newark, New Jersey 07103

The metabolism of ⁷-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, ⁷-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.

Supplementary key words ßbeta;-sitosterol • gas-liquid chromatography • mass spectrometry • silver nitrate thin-layer chromatography • reversed-phase thin-layer chromatography • two-pool model • ³H/¹⁴C isotope ratio • specific activity decay curves

Submitted on April 24, 1973
Revised on September 10, 1973
Accepted on February 5, 1974

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