Document Detail

The metabolism of cholestanol, cholesterol, and bile acids in cerebrotendinous xanthomatosis.
MedLine Citation:
PMID:  4355999     Owner:  NLM     Status:  MEDLINE    
The metabolism of cholesterol and its 5-dihydro derivative, cholestanol, was investigated by means of sterol balance and isotope kinetic techniques in 3 subjects with cerebrotendinous xanthomatosis (CTX) and 11 other individuals. All subjects were hospitalized on a metabolic ward and were fed diets practically free of cholesterol and cholestanol. After the intravenous administration of [1,2-(3)H]cholestanol, the radioactive sterol was transported and esterified in plasma lipoproteins in an identical manner to cholesterol. In these short-term experiments, the specific activity-time curves of plasma cholestanol conformed to two-pool models in both the CTX and control groups. However, cholestanol plasma concentrations, total body miscible pools, and daily synthesis rates were two to five times greater in the CTX than control individuals. The short-term specific activity decay curves of plasma [4-(14)C]cholesterol also conformed to two-pool models in both groups. However, in the CTX subjects the decay was more rapid, and daily cholesterol synthesis was nearly double that of the control subjects. Plasma concentrations and the sizes of the rapidly turning over pool of exchangeable cholesterol were apparently small in the CTX subjects, and these measurements did not correlate with the large cholesterol deposits found in tendon and tuberous xanthomas. Despite active cholesterol synthesis, bile acid formation was subnormal in the CTX subjects. However, bile acid sequestration was accompanied by a rise in plasma cholestanol levels and greatly augmented fecal cholestanol outputs. In contrast, the administration of clofibrate lowered plasma cholesterol levels 50% and presumably reduced synthesis in the CTX subjects. Plasma cholesterol concentrations and fecal steroid excretion did not change significantly during this therapy. These findings indicate that the excessive tissue deposits of cholesterol and cholestanol that characterize CTX were associated with hyperactive neutral sterol synthesis. The demonstration of subnormal bile acid formation suggests that defective bile acid synthesis may predispose to the neutral sterol abnormalities.
G Salen; S M Grundy
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  The Journal of clinical investigation     Volume:  52     ISSN:  0021-9738     ISO Abbreviation:  J. Clin. Invest.     Publication Date:  1973 Nov 
Date Detail:
Created Date:  1974-01-05     Completed Date:  1974-01-05     Revised Date:  2010-09-10    
Medline Journal Info:
Nlm Unique ID:  7802877     Medline TA:  J Clin Invest     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  2822-35     Citation Subset:  AIM; IM    
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MeSH Terms
Bile Acids and Salts / metabolism*
Brain Diseases / metabolism*
Carbon Radioisotopes
Cholestanol / administration & dosage,  blood,  metabolism
Cholesterol / administration & dosage,  blood,  metabolism*
Cholestyramine Resin / pharmacology
Clofibrate / pharmacology
Esters / blood
Feces / analysis
Injections, Intravenous
Lipoproteins, HDL / blood
Lipoproteins, LDL / blood
Lipoproteins, VLDL / blood
Middle Aged
Xanthomatosis / metabolism*
Reg. No./Substance:
0/Bile Acids and Salts; 0/Carbon Radioisotopes; 0/Esters; 0/Lipoproteins, HDL; 0/Lipoproteins, LDL; 0/Lipoproteins, VLDL; 10028-17-8/Tritium; 11041-12-6/Cholestyramine Resin; 57-88-5/Cholesterol; 637-07-0/Clofibrate; 80-97-7/Cholestanol

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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