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Updated: Aug 12, 2026

Determination of Fatty Acid Oxidation and Lipogenesis in Mouse Primary Hepatocytes
Published on: August 27, 2015
Phytanic acid alpha-oxidation, new insights into an old problem: a review
Ronald J A Wanders1, Gerbert A Jansen, Matthew D Lloyd
1Laboratory Genetic Metabolic Diseases, Department of Pediatrics/Emma Children's Hospital and Clinical Chemistry, Academic Medical Centre, University Hospital Amsterdam, Room F0-224, P.O. Box 22700, 1100 DE Amsterdam, The Netherlands. r.j.wanders@amc.uva.nl
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.
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