| Phytanic acid: production from phytol, its breakdown and role in human disease. | |
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MedLine Citation:
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PMID: 16799769 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Phytanic acid is a branched-chain fatty acid that accumulates in a variety of metabolic disorders. High levels of phytanic acid found in patients can exceed the millimolar range and lead to severe symptoms. Degradation of phytanic acid takes place by alpha-oxidation inside the peroxisome. A deficiency of its breakdown, leading to elevated levels, can result from either a general peroxisomal dysfunction or from a defect in one of the enzymes involved in alpha-oxidation. Research on Refsum disease, belonging to the latter group of disorders and characterized by a deficiency of the first enzyme of alpha-oxidation, has extended our knowledge of phytanic acid metabolism and pathology of the disease greatly over the past few decades. This review will centre on this research on phytanic acid: its origin, the mechanism by which its alpha-oxidation takes place, its role in human disease and the way it is produced from phytol. |
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Authors:
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D M van den Brink; R J A Wanders |
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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: Cellular and molecular life sciences : CMLS Volume: 63 ISSN: 1420-682X ISO Abbreviation: Cell. Mol. Life Sci. Publication Date: 2006 Aug |
Date Detail:
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Created Date: 2006-08-14 Completed Date: 2006-09-29 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 9705402 Medline TA: Cell Mol Life Sci Country: Switzerland |
Other Details:
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Languages: eng Pagination: 1752-65 Citation Subset: IM |
Affiliation:
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Laboratory of Genetic Metabolic Diseases (F0-224), Academic Medical Centre, University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Aldehyde Oxidoreductases
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metabolism Chondrodysplasia Punctata, Rhizomelic / metabolism Humans Oxidation-Reduction Peroxisomal Disorders / metabolism* Peroxisomes / metabolism Phytanic Acid / metabolism* Phytol / metabolism* Protein Transport Refsum Disease / metabolism |
| Chemical | |
Reg. No./Substance:
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14721-66-5/Phytanic Acid; 150-86-7/Phytol; EC 1.2.-/Aldehyde Oxidoreductases; EC 1.2.1.48/long-chain-aldehyde dehydrogenase |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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