| Peroxisomes, Refsum's disease and the alpha- and omega-oxidation of phytanic acid. | |
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MedLine Citation:
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PMID: 17956234 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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In the present paper, we describe the current state of knowledge regarding the enzymology of the phytanic acid alpha-oxidation pathway. The product of phytanic acid alpha-oxidation, i.e. pristanic acid, undergoes three cycles of beta-oxidation in peroxisomes after which the products, including 4,8-dimethylnonanoyl-CoA, propionyl-CoA and acetyl-CoA, are exported from the peroxisome via one of two routes, including (i) the carnitine-dependent route, mediated by CRAT (carnitine acetyltransferase) and CROT (carnitine O-octanoyltransferase), and (ii) the free acid route, mediated by one or more of the peroxisomal ACOTs (acyl-CoA thioesterases). We also describe our recent data on the omega-oxidation of phytanic acid, especially since pharmacological up-regulation of this pathway may form the basis of a new treatment strategy for ARD (adult Refsum's disease). In patients suffering from ARD, phytanic acid accumulates in tissues and body fluids due to a defect in the alpha-oxidation system. |
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Authors:
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R J A Wanders; J C Komen |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't; Review |
Journal Detail:
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Title: Biochemical Society transactions Volume: 35 ISSN: 0300-5127 ISO Abbreviation: Biochem. Soc. Trans. Publication Date: 2007 Nov |
Date Detail:
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Created Date: 2007-10-24 Completed Date: 2008-02-05 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 7506897 Medline TA: Biochem Soc Trans Country: England |
Other Details:
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Languages: eng Pagination: 865-9 Citation Subset: IM |
Affiliation:
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Genetic Metabolic Diseases Laboratory, Room F0-224, Departments of Clinical Chemistry and Pediatrics, Academic Medical Center, University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands. r.j.wanders@amc.uva.nl |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Biological Transport Humans Hydrolysis Hydroxylation Oxidation-Reduction Peroxisomes / metabolism* Phytanic Acid / metabolism* Refsum Disease / metabolism* |
| Chemical | |
Reg. No./Substance:
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14721-66-5/Phytanic Acid |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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