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Phytanic Acid Metabolism in Health and Disease

Phytanic Acid Metabolism in Health and Disease

Ronald J A Wanders, Jasper Komen, Sacha Ferdinandusse

Biochim Biophys Acta. 2011 Sep;1811(9):498-507.

PMID: 21683154

Abstract:

Phytanic acid (3,7,11,15-tetramethylhexadecanoic acid) is a branched-chain fatty acid which cannot be beta-oxidized due to the presence of the first methyl group at the 3-position. Instead, phytanic acid undergoes alpha-oxidation to produce pristanic acid (2,6,10,14-tetramethylpentadecanoic acid) plus CO(2). Pristanic acid is a 2-methyl branched-chain fatty acid which can undergo beta-oxidation via sequential cycles of beta-oxidation in peroxisomes and mitochondria. The mechanism of alpha-oxidation has been resolved in recent years as reviewed in this paper, although some of the individual enzymatic steps remain to be identified. Furthermore, much has been learned in recent years about the permeability properties of the peroxisomal membrane with important consequences for the alpha-oxidation process. Finally, we present new data on the omega-oxidation of phytanic acid making use of a recently generated mouse model for Refsum disease in which the gene encoding phytanoyl-CoA 2-hydroxylase has been disrupted.

Chemicals Related in the Paper:

Catalog Number	Product Name	Structure	CAS Number	Price
AP14721665	Phytanic acid		14721-66-5	Price

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