| Metabolism of the cholestanol precursor cholesta-4,6-dien-3-one in different tissues. | |
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MedLine Citation:
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PMID: 3676336 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Cerebrotendinous xanthomatosis (CTX) is a lipid storage disease where the basic defect is a lack of the mitochondrial C27-steroid 26-hydroxylase involved in bile acid synthesis (EC 1.14.13.15). Cholestanol and cholesterol accumulate in all tissues. At least part of the accumulation of cholestanol is due to a 7 alpha-dehydroxylation of early bile acid intermediates. Cholesta-4,6-dien-3-one, a proposed intermediate in this pathway, is found in increased concentrations in serum of the patients. This study shows that cholesta-4,6-dien-3-one may be metabolized to 4-cholesten-3-one and cholestanol by liver, adrenals and brain. No conversion was found in intestinal mucosa or in kidneys. The capacity to convert cholesta-4,6-dien-3-one into 4-cholesten-3-one and cholestanol varied in different tissues as well as in different species. The results are discussed in relation to the cholestanol accumulation in CTX. |
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Authors:
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M S Buchmann; I Björkhem; S Skrede |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Biochimica et biophysica acta Volume: 922 ISSN: 0006-3002 ISO Abbreviation: Biochim. Biophys. Acta Publication Date: 1987 Nov |
Date Detail:
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Created Date: 1987-12-28 Completed Date: 1987-12-28 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 0217513 Medline TA: Biochim Biophys Acta Country: NETHERLANDS |
Other Details:
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Languages: eng Pagination: 111-7 Citation Subset: IM |
Affiliation:
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Institute of Clinical Biochemistry, Rikshospitalet, University of Oslo, Norway. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Animals Cholestanols / biosynthesis* Cholestenes / metabolism* Cholestenones / metabolism* Chromatography, High Pressure Liquid Female Humans Kinetics Microsomes / metabolism* Microsomes, Liver / metabolism Organ Specificity Rats Rats, Inbred Strains Tritium |
| Chemical | |
Reg. No./Substance:
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0/Cholestanols; 0/Cholestenes; 0/Cholestenones; 10028-17-8/Tritium; 566-93-8/4,6-cholestadien-3-one |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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