Transformation of 4-cholesten-3-one and 7 alpha-hydroxy-4-cholesten-3-one into cholestanol and bile acids in cerebrotendinous xanthomatosis

Journal Article · Wed Aug 01 04:00:00 EDT 1984 · Gastroenterology; (United States)

OSTI ID:6223578

Salen, G; Shefer, S; Tint, G S

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.

• Research Organization: Veterans Administration Hospital, East Orange, NJ
• OSTI ID: 6223578
• Journal Information: Gastroenterology; (United States), Journal Name: Gastroenterology; (United States) Vol. 87:2; ISSN GASTA
• Country of Publication: United States
• Language: English

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59 BASIC BIOLOGICAL SCIENCES
BILE ACIDS
BIOCHEMISTRY
BIOLOGICAL PATHWAYS
BODY
CARBON 14 COMPOUNDS
CARBOXYLIC ACIDS
CELL CONSTITUENTS
CHEMISTRY
CHOLESTEROL
CHOLIC ACID
DIGESTIVE SYSTEM
DISEASES
GLANDS
HYDROXY COMPOUNDS
ISOTOPE APPLICATIONS
LABELLED COMPOUNDS
LIVER
METABOLIC DISEASES
MICROSOMES
ORGANIC ACIDS
ORGANIC COMPOUNDS
ORGANOIDS
ORGANS
PATIENTS
STEROIDS
STEROLS
TRACER TECHNIQUES
TRITIUM COMPOUNDS