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Correspondence to:
Nanda M. Verhoeven.
The relationship between peroxisomal and mitochondrial oxidation of the methyl branched fatty acids, phytanic acid and pristanic acid, was studied in normal and mutant human skin fibroblasts with established enzyme deficiencies. Tandem mass spectrometry was used for analysis of the acylcarnitine intermediates. In normal cells, 4,8-dimethylnonanoylcarnitine (C11:0) and 2,6-dimethylheptanoylcarnitine (C9:0) accumulated after incubation with either phytanic acid or pristanic acid. These intermediates were not observed when peroxisome-deficient cells from Zellweger patients were incubated with the same compounds, pointing to the involvement of peroxisomes in the formation of these acylcarnitine intermediates. Similar experiments with fibroblasts deficient in carnitine palmitoyltransferase I, carnitine-acylcarnitine translocase or carnitine palmitoyltransferase II revealed that mitochondrial carnitine palmitoyltransferase I is not required for the oxidation of phytanic acid or pristanic acid, whereas both carnitine-acylcarnitine translocase and carnitine palmitoyltransferase II are necessary.
These studies demonstrate that both phytanic acid and pristanic acid are initially oxidized in peroxisomes to 4,8-dimethylnonanoyl-CoA, which is converted to the corresponding acylcarnitine (presumably by peroxisomal carnitine octanoyltransferase), and exported to the mitochondrion. After transport across the mitochondrial membrane and transfer of the acylgroup to coenzyme A, further oxidation to 2,6-dimethylheptanoyl-CoA occurs.—Verhoeven, N. M., D. S. Roe, R. M. Kok, R. J. A. Wanders, C. Jakobs, and C. R. Roe. Phytanic acid and pristanic acid are oxidized by sequential peroxisomal and mitochondrial reactions in cultured fibroblasts. J. Lipid Res. 1998. 39: 66–74.
Supplementary key words:
tandem mass spectrometry, peroxisome, mitochondrion, carnitine palmitoyltransferase
Copyright © 1998 by Lipid Research, Inc.
Original Article
Phytanic acid and pristanic acid are oxidized by sequential peroxisomal and mitochondrial reactions in cultured fibroblasts
Nanda M. Verhoevena,
Diane S. Roeb,
Robert M. Koka,
Ronald J. A. Wandersc,
Cornelis Jakobsa, and
Charles R. Roeb
a Department of Clinical Chemistry, Free University Hospital, 1081 HV Amsterdam, The Netherlands
b Institute of Metabolic Disease, Baylor University Medical Center, Dallas, TX
c Departments of Pediatrics and Clinical Chemistry, University of Amsterdam, Academic Medical Center, Amsterdam, The Netherlands
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