Abstract
Phytanic acid (3,7,10,14-tetramethylhexadecanoic acid) is a branched-chain fatty acid which is known to accumulate in a number of different genetic diseases including Refsum disease. Due to the presence of a methyl-group at the 3-position, phytanic acid and other 3-methyl fatty acids can not undergo beta-oxidation but are first subjected to fatty acid alpha-oxidation in which the terminal carboxyl-group is released as CO(2). The mechanism of alpha-oxidation has long remained obscure but has been resolved in recent years. Furthermore, peroxisomes have been found to play an indispensable role in fatty acid alpha-oxidation, and the complete alpha-oxidation machinery is probably localized in peroxisomes. This Review describes the current state of knowledge about fatty acid alpha-oxidation in mammals with particular emphasis on the mechanism involved and the enzymology of the pathway.
Publication types
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Research Support, Non-U.S. Gov't
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Review
MeSH terms
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Amino Acid Sequence
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Animals
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Carbon Dioxide / chemistry
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Carbon-Carbon Lyases / chemistry
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Carbon-Carbon Lyases / metabolism
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Carrier Proteins / chemistry
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Carrier Proteins / metabolism
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Cloning, Molecular
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Formates / chemistry
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Humans
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Liver / metabolism
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Mitochondria, Liver / metabolism
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Mixed Function Oxygenases / genetics
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Mixed Function Oxygenases / metabolism
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Models, Chemical
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Molecular Sequence Data
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Oxidation-Reduction
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Peroxisomes / chemistry
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Peroxisomes / enzymology
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Peroxisomes / metabolism
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Phytanic Acid / chemistry*
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Phytanic Acid / metabolism*
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Receptors, Cytoplasmic and Nuclear / agonists
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Refsum Disease / metabolism
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Transcription Factors / agonists
Substances
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Carrier Proteins
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Formates
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Receptors, Cytoplasmic and Nuclear
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Transcription Factors
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sterol carrier proteins
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formic acid
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Carbon Dioxide
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Phytanic Acid
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Mixed Function Oxygenases
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PHYH protein, human
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Carbon-Carbon Lyases
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2-hydroxyphytanoyl-coenzyme A lyase