| Structure and lipid distribution of polyenoic very-long-chain fatty acids in the brain of peroxisome-deficient patients (Zellweger syndrome). | |
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MedLine Citation:
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PMID: 3435449 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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The polyenoic fatty acids with carbon chain lengths from 26 to 38 (very-long-chain fatty acids, VLCFA) previously detected in abnormal amounts in Zellweger syndrome brain have been shown to be n-6 derivatives and therefore probably derived by chain elongation of shorter-chain n-6 fatty acids such as linoleic acid and arachidonic acid. Polyenoic VLCFA are also present in Zellweger syndrome liver, but this tissue differs significantly from brain in that the saturated and mono-unsaturated derivatives are the major VLCFA. Zellweger syndrome brain polyenoic VLCFA are present in the neutral lipids predominantly in cholesterol esters, with smaller amounts in the non-esterified fatty acid and triacylglycerol fractions. These fatty acids are barely detectable in any of the major phospholipids, but are present in significant amounts in an unidentified minor phospholipid. The polyenoic VLCFA composition of this lipid differs markedly from that observed for all other lipids, as it contains high proportions of pentaenoic and hexaenoic fatty acids with 34, 36 and 38 carbon atoms. A polar lipid with the chromatographic properties in normal brain contains similar fatty acids. It is postulated that the polyenoic VLCFA may play an important role in normal brain and accumulate in Zellweger syndrome brain because of a deficiency in the peroxisomal beta-oxidation pathway, although a possible peroxisomal role in the control of carbon-chain elongation cannot be discounted. |
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Authors:
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P Sharp; A Poulos; A Fellenberg; D Johnson |
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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: The Biochemical journal Volume: 248 ISSN: 0264-6021 ISO Abbreviation: Biochem. J. Publication Date: 1987 Nov |
Date Detail:
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Created Date: 1988-03-09 Completed Date: 1988-03-09 Revised Date: 2009-11-18 |
Medline Journal Info:
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Nlm Unique ID: 2984726R Medline TA: Biochem J Country: ENGLAND |
Other Details:
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Languages: eng Pagination: 61-7 Citation Subset: IM |
Affiliation:
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Department of Chemical Pathology, Adelaide Children's Hospital, South Australia. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Brain Chemistry* Cholesterol Esters / analysis Chromatography, Gas Fatty Acids, Unsaturated / analysis* Humans Lipids / analysis* Liver / analysis Microbodies Nerve Degeneration* Syndrome Tissue Distribution |
| Chemical | |
Reg. No./Substance:
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0/Cholesterol Esters; 0/Fatty Acids, Unsaturated; 0/Lipids |
| Comments/Corrections | |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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