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Title Transformation of 4-cholesten-3-one and 7 alpha-hydroxy-4-cholesten-3-one into cholestanol and bile acids in cerebrotendinous xanthomatosis
Creator/Author Salen, G. ; Shefer, S. ; Tint, G.S.
Publication Date1984 Aug 01
OSTI IdentifierOSTI ID: 6223578
Other Number(s)CODEN: GASTA
Resource TypeJournal Article
Resource RelationGastroenterology ; Vol/Issue: 87:2
Research OrgVeterans Administration Hospital, East Orange, NJ
Subject550201 -- Biochemistry-- Tracer Techniques ;550501 -- Metabolism-- Tracer Techniques; ;CHOLESTEROL-- BIOLOGICAL PATHWAYS;METABOLIC DISEASES-- BIOCHEMISTRY; BILE ACIDS;CARBON 14 COMPOUNDS;CHOLIC ACID;LIVER;MICROSOMES;PATIENTS;TRACER TECHNIQUES;TRITIUM COMPOUNDS
Related SubjectBILE ACIDS;BODY;CARBOXYLIC ACIDS;CELL CONSTITUENTS;CHEMISTRY;DIGESTIVE SYSTEM;DISEASES;GLANDS;HYDROXY COMPOUNDS;ISOTOPE APPLICATIONS;LABELLED COMPOUNDS;ORGANIC ACIDS;ORGANIC COMPOUNDS;ORGANOIDS;ORGANS;STEROIDS;STEROLS
Description/Abstract In order to determine whether cholestanol and bile acids are derived from the same precursor, key intermediates of both biosynthetic pathways beyond cholesterol were administered intravenously to a patient with cerebrotendinous xanthomatosis and to a control subject.^After pulse-labeling with (4-/sup 14/C)4-cholesten-3-one and (G-/sup 3/H)7 alpha-hydroxy-4-cholesten-3-one, cholestanol, cholesterol, and the two primary bile acids, cholic acid and chenodeoxycholic acid were isolated from specimens of bile.^In other studies, the in vitro formation of 4-cholesten-3-one from cholesterol was measured in hepatic microsomal fractions prepared from a subject with cerebrotendinous xanthomatosis and from 3 control individuals.^In all subjects, cholic acid and chenodeoxycholic acid were labeled with tritium, but neither cholesterol nor cholestanol contained this isotope.^In contrast, /sup 14/C was detected in the cholestanol fraction with trace amounts in chenodeoxycholic acid, cholic acid, and cholesterol.^The results indicate that 4-cholesten-3-one was converted primarily into cholestanol and 7 alpha-hydroxy-4-cholesten-3-one into cholic acid and chenodeoxycholic acid.^Neither ketonic steroid was transformed into cholesterol.^The increased production of cholestanol in cerebrotendinous xanthomatosis may be accounted for by enhanced hepatic formation of 4-cholesten-3-one.^7 alpha-Hydroxy-4-cholesten-3-one is a precursor of bile acids, but not of cholestanol.
Country of PublicationUnited States
LanguageEnglish
FormatPages: 276-283
System Entry Date2001 May 13

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