ω-Hydroxylation of phytanic acid in rat liver microsomes

The 3-methyl-branched fatty acid phytanic acid is degraded by the peroxisomal α-oxidation route because the 3-methyl group blocks β-oxidation. In adult Refsum disease (ARD), peroxisomal α-oxidation is defective, which is caused by mutations in the gene coding for phytanoyl-CoA hydroxylase in the maj...

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Bibliographic Details
Main Authors: J.C. Komen, M. Duran, R.J.A. Wanders
Format: Article
Language:English
Published: Elsevier 2004-07-01
Series:Journal of Lipid Research
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S0022227520317909