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- Title
Phytanic acid: production from phytol, its breakdown and role in human disease.
- Authors
van den Brink, D M; Wanders, R J A
- Abstract
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.
- Publication
Cellular and molecular life sciences : CMLS, 2006, Vol 63, Issue 15, p1752
- ISSN
1420-682X
- Publication type
Journal Article
- DOI
10.1007/s00018-005-5463-y